You are on page 1of 186

..............................................................

:




: : :
:


haemoglobin 13 male
12 female
stages of RBCs development
stem cells proerythroblast erythroblast
normoblast Reticulocyte mautre RBCs
normo blast normo blast
iron Normoblast iron
reticulocyte

stages peripheral blood


reticulocyte mature RBCs
bone marrow peripheral blood
mature RBCs reticulocyte
reticulocyte about 0.2-2% Percent

Peripheral blood reticulocytosis
bone marrow hyper active hyper active
RBCs precursors
1


..............................................................
bone marrow RBCs
bone marrow RBCs
Reticulocytes
hemolysis hemorrhage bone
marrow excessive production of RBCs
Reticulocytes
iron deficiency
anemia B12 deficiency
bone marrow
mature and reticulocytes
iron deficiency anemia
bone marrow normoblast
mature RBCs ......
Iron therapy bone marrow
reticulocytes RBCS
Reticulocytosis

MCQ
reticulocytosis
MCQ reticulocytes
reticulocytosis bone marrow activity
reticulocytosis aplastic anemia
bone marrow aplasia
reticulocytosis
reticulocytosis iron deficiency
anemia
hemolysis
hemorrhage
aplastic anemia
reticulocytes Peripheral blood
2


..............................................................
haemoglobin A
haem iron and prtoporphyrin
globin alpha Beta
alpha haemoglobin F

:
ESR

anticoagulant
RBCs
column of plasma
.....
RBCs Column of plasma
factors ESR
RBCs rouleaux
RBCs .... rouleaux
immunoglobulins fibrinogen
fibrinogen immunoglobulin

Infection Inflammatory
Inflammatory infection
Gamma globulin fibrinogen
RBCs
RBCs orders
RBC repulsion
Inflammation Infection
immunoglobulin fibrinogen ESR

ESR

3


..............................................................
Westergren method anti coagulant
Na citrate
Wintrobe method EDTA

haematocrit value

RBCs

volume of RBCs total blood volume


haematocrit value
haematocrit value assesment
Na
citrate
true accurate

Westergren method Wintrobe method

Wintrobe
haematocrit value haematocrit value
RBCs in relation to blood volume
Na citrate diluation false
results
4


..............................................................
causes of moderate rise of ESR
infection , anemia , myocardial infraction
myocardial infraction ESR
pulmonary infraction
pulmonary embolism Pulmonary infraction
rheumatoid ESR
ESR
tuberculosis Pyogenic infection
malignancy Leukaemia Lymphoma Multiple
myeloma
pyogenic infection

ESR
leukaemia Lymphoma
ESR normal
polycythaemia
polycythaemia rubra vera

polycythaemia RBCs

ESR
RBCs

anemias 2 ESR
sickle cell and spherocytosis

RBCs spherocytes sickle cell
rouleaux
MCQ
5


..............................................................

haematocrit value
packed RBCs
Packed RBCs volume of RBCs blood volume
RBCs volume plasma volume
RBCs volume total blood volume
male
female

polycythaemia
haematocrit value MCQ

..... .....
haematocrit value
normal
...
fluids
Interstitial tissues Intravascular




Interstitial tissue intravascular
volume
haematocrit value
haematocrit value Immediate haemorrhage
hamorrhage

peptic ulcer varices
hematemesis ....
6


..............................................................

Haematocrit value
) ( melena


haematocrit value
haematocrit value
patient recurrent bleeding follow
up
how can you follow this patient
follow patient haemoglobin RBCs
haematocrit value
provided base line haematocrit
value
...
diluate

Blood indices
mean corpuscular haemoglobin
RBC
RBC RBCs


average 32-27
RBC
blood index
mean corpuscular volume
packed cell volume packed cell
volume RBCs
RBC RBCs RBCs

Mean corpuscular volume -78
7


..............................................................
98
100 macro
Mean corpuscular volume blood index
normal normocytic
macro cytic
microcytic

8 problem

blood indices

... ...... anemic


mean corpuscular volume

normocytic , macrocytic and microcytic

morphological classification according


to blood indices especially mean
corpuscular volume
microcytic anemia
iron deficiency anemia
normo cytic aplastic anemia , hemolytic
macrocytic

blood indices


..............................................................
mean corpuscular haemoglobin concentration
percent RBCs

packed cell volume
in relation RBCs volume
30
33
mean corpuscular haemoglobin concentration
low normal

No increase in mean corpuscular haemoglobin


concentration

normochromic
Hypochromic Hyperchromic
.... Hyperchromic 13 15
2009

hyperchromia
mean corpuscular haemoglobin concentration

RBC
hyperchomic

spherocytosis spheroctye mean corpuscular
volume
concentration relatively high
exception

spherocytosis
9


..............................................................

hyper chromia
Mean corpuscular haemoglobin concentration High
relatively in spherocytosis

blood film

blood film
Oral blood film
microcytosis , macrocytosis , hypochromia
anisocytosis variation of the size of the
RBCs
Poikilocytosis variation of the shape of the RBCs
anisocytosis poikilocytosis non
specific
deficiency
deficiency RBCs RBCs

variation size shape
punctuate basophilia basophilic
RBCs lead poison
Howell Jolly bodies
parasites in RBCs malaria Problem
solving blood film Infection
parasite malaria
shape of the RBCs
written
Burr cell Echinocyte
.... uremia Burr cells

Stomatocytes
10


..............................................................
target cells Codocyte
Codocyte ..... target Codocyte
iron deficiency , liver disease, Thalassaemia
Acanthocyte RBCs chronic liver
disease
RBCs .... RBCs

.....
spleen .... RBCs RBCs
...

spleen
post splenoectomy Acanthocyte
Acanthocyte advanced chronic liver
disease
Sickle cell lancet cell
spherocyte spherocytosis
Schistocytes RBCs fragmented Helmet
cell shcizocyte

Tear drop cell Dacrocyte
myelofibrosis
(Red cell distribution width (RDW
varitation size
RBCs

... .... variation

variation %15
accepted
11


..............................................................

variation %50

) variation %80
(
anisocytosis RBCs RBCs
variation size
Red cell distribution width
((RDW
Red cell distribution width
variation normally % 14.511.5
variation
deficiency iron deficiency B12
deficiency ....

Iron therapy
iron ....ferrous sulfate 200 mg tab ferrous
gluconate 300 mg tab doses
iron nausea
nausea
iron

absorption
irritation
iron Low concentration
iron so irritant

iron absorption ascorbic acid

iron intolerance
12


..............................................................

nausea vomiting abdominal pain

plz

stool dark stool
dark
respond
reticulocytosis
bone marrow Normo blast
reticuolcyte RBCs
response clinical
failure of oral iron :
wrong diagnosis thalassaemia
minor
thalassaemia minor hypochromic
microcytic iron deficiency anemia
female 10 iron therapy
3
thalassaemia minor
thalassaemia minor
10-9.5
iron deficiency thalassaemia minor
haemoglobin electrophoresis


malabsorption
chronic infection
chronic infection failure of iron utilization
cytokines Infection
cytokines bone marrow iron

13


..............................................................
parenteral iron
.... Iron Saccharated iron
((ferrosac
side effects Minimal ferrosac

iron dose
iron intermittent IV infusion
Iron deficiency anemia

ferrosac 100
50 100 Intra venous
every other day

any rare
anaphylaxis


ferrosac
hydrocortisone

Anaemia
definition
decrease haemoglobin and RBCs count
in relation to age and sex
haemoglobin
adult child
morphological
.... mainly blood indices
.. anaemia oxygen carrying capacity
14


..............................................................
tissues Hypoxia
hypoxia tissues
compensatory mechanism
vasodilation tissue perfusion cardic output

3DPG-2 haemoglobin

8 oxygen
tissues 10
carrdic output vaso dilation
chronic anaemia
...
7 chronic
anaemia
....
....
anaemic chronic anaemia
actue anaemia
anaemia acute few hours
hemolytic crisis G6PD
11 4
anaemic heart failure
symptoms signs anaemia
fatigue headache
hemic murmur

heart failure anaemic heart failure Heart
failure high cardic output failure
cardic output
tissue blood oxygen quality
oxygen
heart failure
digitalis .... haemoglobin
15


..............................................................
problem solving
anaemia clinical examination
anaemia
anaemia ... iron deficiency

specific signs of different types of
anaemias

koilonychias in iron deficiency anaemia
Jaundice splenomegaly hemolytic
neurological subacute combined
degeneration B12 deficiency ...................

iron deficiency anaemia


iron %10
iron
10 iron 1

iron kinetics
oral iron ferric HCL
ferrous ferrous form of absorption
small intestine
small intestine apoferritin ferritin
system
apoferritin ferritin iron
ferritin stores
delivery iron transferrin
transferrin globulin iron
iron serum iron
... serum iron serum iron
transferrin free iron blood
free iron in blood injurious tissues
haemoglobin transferrin
16


..............................................................
serum iron transferrin
iron binding capacity transferrin
iron ..... iron binding capacity

) (
iron level iron level serum
iron transferrin ratio iron
binding capacity transferrin
transferrin saturation
transferrin saturation iron transferrin
Most accurate iron

serum iron iron transferrin


iron binding capacity
ratio
most accurate transferrin saturation
Investigation
reflect iron
transferrin ....
bone marrow ... bone marrow
receptor bone marrow RBC
normoblast
normoblast iron reticulocyte mature
RBC periphral blood
serum ferritin reflect iron stores
serum ferritin accurate phase reactant

17


..............................................................
platelets phase reactant serum ferritin phase
reactant
platelets Infection Inflammatory diseases
ferritin

aetiology of iron deficiency anaemia


Iron deficiency anaemia adult chronic
blood loss
blood loss GIT bleeding GIT

vaginal bleeding utrine bleeding in female
menstruation problems

Ankylostoma anaemia iron
deficiency anaemia


microcytic hypochromic approach
microcytic hypochromic iron deficiency
anaemia commonest anaemia
thalassaemia
Iron profile haemoglobin electrophoresis

general features iron deficiency anaemia
koilonychia spooning
Loss of luster
cause GIT bleeding utrine bleeding

diagnosis

anaemia Low
mean
18


..............................................................
corpuscular volume microcytic hypochromic
... microcytic hypochromic iron deficiency
anaemia .... iron profile
serum iron ... iron binding capacity ...
serum ferritin ... transferrin saturation
iron RBC bone marrow
protoporphyrin haemoglobin
.... protoporphyrin free
protoporphyrin RBC Iron deficiency
Protoporphyrin
bone marrow
examination
bone marrow examination diagnostic
confirm
diagnosis bone marrow
anaemic microcytic hypochromic iron
profile

bone marrow examination absolutely indicated

hyper plasia bone marrow
bone marrow normoblast
normoblast bone marrow iron

anaemia hypochromic microcytic
iron deficiency
Iron deficiency anaemia

upper GIT endoscope Lower endoscope



eosinophilia
occult blood in stool not reliable 100% occult
19


..............................................................
blood
Molecules stool
occult blood in stool RBCs
stool occult blood in stool RBCs
chemical reaction
haemoglobin stool
iron loss blood loss
3 false results
Occult blood in stool positive GIT bleeding
occult blood in stool Positive false

iron

megaloblastic Anaemia
mean corpuscular volume 100
115
B12
B12 .... strictly
vegetarian liable
B12 B12 intrnisic factor
small intestine
terminal ileum
terminal ileum
terminal ileum deficiency
terminal ileum B12 deficiency
reaction
homocysteine amino acids
injury endothelium
) coronary (
lipid profile hypertensive
diabetic !!!!
20


..............................................................
risk factor
homocysteine
B12 homocysteine
methionine
Homocysteine B12 folic

folic acid B12 atherosclerosis
) ( antioxidant
B12 folic acid
B12 folic B12
folic iron calcium


quality of life

homocysteine
atherosclerosis
folic
FIGLU test
clinical pathology
histidine FIGLU FIGLU glutamic
acid folic acid
folic acid deficiency FIGLU


B12 Folic acid serum
Causes of megaloblastic anaemia
causes of iron deficiency anaemia
adult chronic blood loss
B12 deficiency B12 deficiency
intrinsic factor small
intestine
21


..............................................................

B12 stores
B12
stores liver
folic acid
very easy Increase demand ..
iron deficiency folic acid deficiency B12
B12

chronic gastritis
chronic gastritis A and B
A auto immune
B bacterial
auto immune
pernicious antibody
parietal cells anti body intrinsic factor
B Helicobacter pylori
small intestine
malabsorption
Diphyllobothrium latum
B12
folic acid deficiency

iron
anti folate
anti folate
trimethoprim antifolate
sulpha

22


..............................................................
pernicious anaemia
B12 deficiency auto immune disease

pernicious anaemia B12 deficiency


pernicious anaemia B12 deficiency
auto immune disease
parietal cells intrinsic factor
anti parietal cell anti body anti intrinsic factor anti
body

associated with other auto immune
disease myxoedema , thyroiditis , vitilligo
pathophysiology of megaloblastic
anaemia

folic acid B12 maturation
nucleus cell division
Miosis Mitosis
nucleus development
cytoplasm division


division
nuclear material
folic acid and B12
cytoplasm nucleus ....
RBCs bone marrow RBCs
nucleus well developed
cytoplasm RNA
DNA
bone marrow
bone marrow bone marrow
megaloblastic changes
megalobastic changes bone marrow

23


..............................................................
megaloblastic anaemia
Megaloblastic changes bone marrow
deficiency folic acid B12
RBCs
RBCs megaloblast bone marrow
bone marrow
Peripheral blood blood film
bone marrow intra medullary
hemolysis


B12 deficiency jaundice
jaundice Intra medullary hemolysis
Jaundice bilirubin

platelets
RBCs
B12 deficiency folic acid deficiency pan
cytopenia

Non megaloblastic macrocytosis
RBCs peripheral blood
bone marrow .... normal ... non megaloblastic

hypothyroidism alcohol excess chronic liver
disease


RBCs Lipid RBCs
RBCs .... RBCs
macrocyte B12 folic acid deficiency
bone marrow
macrocytic non megaloblastic
) (

24


..............................................................
... anaemic
mean corpuscular volume 120


macrocytosis megaloblastic nonmegaloblastic
megaloblastic folic acid B12
non megalo blastic chronic liver disease



RBCs peripheral blood
reticulocytes mature RBCs

reticulocyte
reticulocytosis hemolysis
macrocytosis

hemolytic crisis macrocytosis
, reticulocytosis reticulocyte


clinical pictures
B12
anaemia GIT manifestations subacute combined
degeneration

iron deficiency B12 written problem


solving
macrocytosis megaloblastic
non megaloblastic
neuro neuro
25


..............................................................

pallor fatigue mean
corpuscular volume ...
... examination data
glove and stock hypothesia bilateral
exaggerated knee jerk
pyramidal glove and stock
glove and stock ankle jerk Lost
peripheral neuropathy
subacute combined degeneration bilateral
pyramidal
hyper reflexia ... peripheral
neuropathy lost ankle knee jerk
exaggerated

...
mean corpuscular volume neuro
sub acute combined degeneration

investigation
anaemia
anaemia


... blood indices mean corpscular volume
mean corpuscular volume .... Macro cytic
megaloblastic non megaloblastic
megaloblastic folic B12
bone marrow examination mandatory
mandatory
bone marrow megaloblast
non megaloblast RBCs
peripheral blood alcoholic cirrhotic
hypothyroidism reticulocytosis
26


..............................................................
B12
B12
stomach small intestine

mean corpuscular volume B12
intrinsic factor
stomach small intestine test
Schilling test
Schilling test
vitamin B12 injection stores
B12 oral
B12
digestion and absorption blood
blood stores
B12 absorption
Intrinsic factor small intestine
B12 oral
intestine

intrinsic factor .... stomach
small intestine
other findings
MCQ
poikilocytosis anisocytosis non specific
anisocytosis S variation in size

poikilocytosis p variation in shape
Howell jolly bodies nuclei well
developed megaloblast
nuclear material developed remnant
Howell jolly bodies
27


..............................................................
WBCs platelets
bilirubin
reticulocyte
Hemolysis macrocytosis


Hemolysis macrocytosis
.... folic acid deficiency hemolysis



hemolysis bone marrow
proliferating consume folic acid folic
acid deficiency
hemolysis folic
acid B12 iron therapy
hemolytic iron over load

300-200
...
thalassaemia thalassaemia
supplement
folic acid B12 iron contra
indicated

iron therapy
iron contra indicated

28


..............................................................



hemolysis depletion folic acid
macrocytosis

hemolytic crisis reticulocytosis
hemolytic crisis reticulocytosis
reticulocytes macrocytes

hemolytic anemia
megaloblastic anaemia hemolysis
Intramedullary

hemolytic anaemia folic acid


hemolysis
folic acid supplement
hemolytic anaemia crisis
reticulocytosis reticulocyte
B12 deficiecny Intra medullary
hemolysis
bilirubin

WBCs showing hypersegmented
neutrophils neutrophils hyper segmented
hyper segmented WBCs
division nuclear material
development of the neutrophils
29


..............................................................
nucleus hypersegmented
nucleus
nucleus Hypersegmentation .

hyper segmented neutrophils MCQ
B12 or folic acid deficiency

platelets
folic acid B12


Three parasites give different types of anaemia
Ankylostoma iron deficiency anaemia
eosinophilia
Diphyllobothrium latum vit B12
megaloblastic anaemia
malaria haemolytic anaemia
para sites anaemia
diagnosis treatment of each
parasite dose duration of the drug
ankylostoma diphyllobothrium latum
malaria


pancytopenia written written
anaemic data
platelets RBCs WBCs case of
pancytopenia
pancytopenia

30


..............................................................
aplastic anaemia
hypersplenism
B12 folic acid deficiency

Sidroblastic anaemia

sidroblastic anaemia
MCQ ... bone marrow
normoblast
Normoblast reticulocyte mature
RBCs
Normoblast normoblast
.... iron protopophyrin
protoporphyrin Iron haem
...
defect
protoporphyrin
iron normoblast bone marrow
protoporphyrin
sidroblast
iron granules normoblast
protoporphyrin
sidrosis
normoblast sidroblast
sidroblast normoblast
protoporphyrin

iron bone marrow
normoblast

normoblast iron ........
31


..............................................................
protoporphyrin
protoporphyrin
normoblast iron
sidrosis
sidroblast normoblast

sidroblast
iron granules bone marrow normoblast
precipitated

sidroblast pericipitated iron granules


sidroblast bone marrow
normoblast
definite diagonis of sidroblastic anaemia
bone marrow examination

microcytic anaemia sidroblastic anaemia

iron ..... RBCs
normoblast
haemoglobin protoporphyrin iron
haemoglobin
peripheral blood bone marrow RBCs
iron deficiency anaemia hypochromic microcytic


bone marrow
iron protoporphyrin absolutely
bone marrow sidroblast
bone marrow
defect partial bone marrow
32


..............................................................
iron protoporphyrin
haemoglobin
RBCs bone marrow iron deficiency
microcytic hypochromic

blood indices hypochromic microcytic iron
profile iron deficiency
haemoglobin electrophoresis thalassaemia
minor
sidroblastic anaemia
bone marrow examination
sidroblast


RBCs peripheral blood iron
protoporphyrin haemoglobin
iron
microscope iron granules precipitated
RBCs peripheral blood
iron granules pappenheimer bodies

causes

acquired
INH
iron
protoporphyrinvitamin B6
INH antagonize vit B6 sidroblastic

INH vitamin B6
vitamin B12 and folic acid Megaloblastic
33


..............................................................
anaemia
Vitamin B6 ...
sidroblastic anaemia
pappenheimer bodies Iron
granules peripheral blood
blood film pappenheimer iron granules
sidroblastic anaemia

stop drug vitamin B6
Just sidrobalstic anaemia
Normoblast iron molecules
bone marrow RBCs peripheral blood
iron deficiency anaemia
pappenhemier bodies
patient with peptic ulcer GIT
bleeding black stool melena iron therapy
peptic ulcer
) ( DE-
NOL
peptic
ulcer
helicobacter

leucocyte
myeloid line lymphoid line
myeloid line
myeloid stem cells
Myeloid stem cells myeloblast
promyelocyte myelocyte metamyelocyte
neutrophils
blast
34


..............................................................
bone marrow
promyelocyte , myelocyte and metamyelocyte
more developed
mature neutrophils

lymphoid stem cells


lymphocyte prelymphocyte lymphoblast
lecocytosis ....
Mature neutrophils
Mature lymphocyte
proliferation leukaemia leukaemia
Immature stages of WBCs
myeoblast Myeloid line
Acute myeloid leukaemia
neoplasm lymphoid line
Acute lymphoblastic leukaemia lymphoblast
chronic pre-lymphocyte leukaemia
lymphocytic leukaemia
metamyelocyte promyelocyte leukaemia
chronic myeloid leukaemia
proliferation blasts acute
mainly
.... blasts mature chronic
blasts
blasts aggressive
lymphoblast proliferation
bone marrow Myeloblast
35


..............................................................
bone marrow failure Infiltration
myeloblast
blasts Acute myeloblastic leukaemia
bone marrow
neutrophils neutrophils
platelets RBCs blasts
neutrophils neutropenia
bone marrow anaemia , thrombocytopenia
blasts peripheral blood blasts
acute myeloblastic Acute myeloid leukaemia
leukaemia
Acute lymphoblastic leukaemia
bone bone marrow lymphoblast
lymphocyte mature marrow
platelets RBCs lymphoblast
early bone marrow failure
acute leukaemia
bone marrow failure
proliferation blasts
normal WBCs bone marrow
platelets RBCs
chronic leukaemia

late bone marrow failure
more or less chronic

bone marrow failure
so late
behavior leucocytes
bone marrow proliferation leucocytes
mature RBCs platelets
WBCs
36


..............................................................
thrombocytopenic anaemic
Infection mature WBCs
WBCs RBCs
WBCs Tissue invasion circulation
bone lung Liver spleen
lymph nodes skin
organs Infiltrated WBCs
disturbances
functions
Leukaemia mortality
Anaemia infection hemorrhage
Inflitration vital organs
bone marrow failure
Acute chronic

immature WBCs normally % 3


Immature WBCs
Immature promyelocyte blasts
blast bone marrow

shift to the left Leucocytosis


Immature WBCs

bone marrow
bone marrow WBCs
bone marrow
leucocytosis WBCs Immature
bone marrow WBCs
infection, bleeding and hemolysis
bleeding hemolysis
RBCs
bone marrow proliferate
.... platelets
37


..............................................................
..... WBCs
bone marrow emergency
Infection emergency bone marrow
Infection RBCs
WBCs platelets
RBCs Infection
Infection cytokines
bone marrow RBCs
hemolysis
hemolysis leucocytes platelets
RBCs bone marrow proliferation
hemolysis hemorrhage
shift to the left
shift to the left WBCs
immature
Immature WBCs
staff staph aureus
staff s , t , a, double f
staff cell staff cells immature neutrophils
staff .......immature neutrophils
shift to the left bone marrow
...

leucocytosis with shift to the left
shift to the right
shift to the right
folic acid and B12 deficiency shift to the right
leucocytosis
Leucopenia neutrophils hyper
segmented

38


..............................................................
B12 and folic acid deficiency
shift to the right

leucopenia neutrophils hypersegmented

leucocytosis Immature
immature
staff cells
staff cells
immature neutrophils
character
nucleus not well developed nucleus
segment two segments
neutrophils segments

segments Neutrophils staff cells
immature
hyper segmented folic acid and B12
deficiency
MCQ clinical pathology
shift to the left ..... leucocytosis
immature neutrophils
immature neutrophils staff cells
Infection hemolysis
Bleeding
Infection ... Infection
shift to the left infection bleeding or hemolysis
toxic granulations
toxic granulations
toxic granulation sign neutrophils
) (
blood blood
blood clinical pathology 75
39


..............................................................
40 blood
) ( blood
blood
blood bloooooooooood
) (
infection toxic granulations
neutrophils sign toxic
granulations
toxic granulaltions cytoplasm
neutrophils vacuoles
infections shift to the left
shift to the left leucocytosis immature WBCs
staff cells
neutrophils Infection
toxic granulations
toxic granulations extremely
specific infection Infection
leukaemia toxic granulations

neutrophils
neuturophilia Neutropenia
absolute count neutrophils 7000-2000
percent absolute count
take care percent absolute count
total leucocytic count 10000
neutrophils 50% neutrophils 50% 10000
absolute count neutrophils 5000
5000 range Normal

neutrophila pyogenic infection
tissue damage
40


..............................................................
myocardial infraction pulmonary infraction
leukaemias
Myeloproliferative rheumatoid
cortisone
neutrophilia neutrophils
wall blood vessels adhesion
adhesion
Neutrophils blood stream
neutrophilia
bone marrow WBCs ....
neutrophils
endothelium blood vessels
Neutrophilia
neutrophilia function neutrophils
Impaired

eosinophils

eosinophilia problem
... neutrophils written oral
eosinophils written oral
neutrophils written 2008
... neutrophils ... ... neutrophilia
neutropenia short
neutrophilia full stop

eosinophils specific
eosinophilia allergy parasite
eosinophilia hydatid disease
Parasite
fasciola

Liver disease eosinophilia
hydatid disease fasciola
fasciola
41


..............................................................

fasciola ... hepatitis like...
hepatitis liver enlarged tender
hepatitis eosinophilia

.N.B amoebic liver eosinophila

eosinophilia
fasciola
fasciola fasciola

fasciola
amoebic liver parasite liver
Liver amoebic liver
amoebic liver eosinophilia
hydatid

fasciola biliary system
Liver parenchyma alkaline phophatase
transaminases general


parasite
addison's disease
addison's Addison's

vasculitis Strug strauss vasculitis


primary hypereosinophilic syndrome
eosinophils very high
42


..............................................................
eosinophils Infiltration Heart Lung vital
organ
hypereosinophilic syndrome
eosinophils
steroid

allergy cortisone addison's
cortisone Hodgkin's cortisone vasculitis
cortisone
cortisone

cortisone
eosinophils lymphocytes

Basophils

myeloproliferative , chronic myeloid ,
myelosclerosis
polycythaemia rubra vera
basophils

lymphocyte
Infection sore thorat
lymphocytosis
lymphocytosis
viral
viral infection T.B. , Brucella and
typhoid
leukaemias
Atypical lymphocytosis
infectious mononucleosis
43


..............................................................
.... Lymphocytosis
chronic lymphocytic leukaemia
infectious mononucleosis

male 65 yrs old . liver , spleen and lymph nodes

persistent lymphocytosis
chronic lymphocytic leukaemia

infectious mononucleosis
yrs 16 liver , spleen and lymph nodes
Lymphocytosis Atypical
lymphocytosis
Lymphocyte
viral infection infectious
mononucleosis
CMV

sore thorat infectious
mononucleosis very common
Lymphopenia
HIV

Monocytes
bone marrow
Peripheral blood tissues tissue
macrophages

value of Bone marrow examination
diagnostic and confirmatory
diagnostic diagnostic
44


..............................................................

bone marrow examination is mandatory

... bone marrow
examination
... multiple myeloma plasma cells
bone marrow
myelofibrosis fibrous tissues bone marrow
A leukaemic leukaemia blasts bone marrow
peripheral blood
aplastic anaemia
sidroblastic anaemia

confirmatory
leukaemia Peripheral blood
A leukaemic leukaemia
leukaemia bone marrow
bone marrow
confirm calssifications
iron deficiency
Megaloblastic
I.T.P bone marrow examination just to
confirm exclude similar condition
I.T.P idiopathic
thrombo cytopenic purpura
exclusion
bone marrow examination
confirm exclude similar condition
45


..............................................................
A leukaemic leukaemia

A leukaemic leukaemia blasts bone marrow


platelets thrombocytopenia
Idiopathic
bone marrow

I.T.P I.T.P



...... bone marrow

almost sure I.T.P bone marrow
sternum aspiration
... dry tap
bone marrow fibrosis
trephine from iliac crest Local
anathesia anterior superior iliac spine


bone
marrow
bone marrow fibrous tissue
myelofibrosis
dry tap Myelofibrosis fibrous tissue
bone marrow

leukaemia peripheral blood Leucocytosis
blasts ... leukaemia Peripheral
blood
Acute leukaemia 100,000 total
leucocytic count blasts
platelets
46


..............................................................
RBCs

... leucocytosis
40,000 leukaemia
leukaemia like
leukemoid oid like

Leukaemia

Infection
leukemoid reaction
leukemoid reaction leukaemia
leukaemia abscess
total leucocytic count may exceed 50,000
usually 50,000
total leucocytic count 100,000
Leukaemia
leukemoid reaction
platelets normal RBCs almost normally

bone marrow normally proliferation

blasts

leucocytes alkaline phosphatase
Normal leucocytes alkaline phosphatase
leucocytes leukaemia ...
leucocytic alkaline phosphatase High
leukemoid reaction
functioning WBCs
immature cells never exceed 5%
blasts
47


..............................................................
.... leukaemia
blood picture leukaemia like Leukemoid
Leucocytois platelets RBCs

blasts bone marrow
leucocytic alkaline phosphatase


blood picture fever

problem fever blood picture
neutrophils ...
lymphocyte ... viral
leukaemias Lymphomas
leucocytosis due to infection
steroid
infection toxic granulations
MCQ
leukoerythroblastic reaction....
blasts WBCs
..... bone marrow blasts WBCs
peripheral blood leukaemia
blasts RBCs bone marrow
leukaemia blasts RBCs

common
leukoerythroblastic blasts
leucocytes blasts RBCs Peripheral
blood
bone marrow bone
48


..............................................................
marrow blasts Peripheral blood

bone marrow infiltration myeloma
leukaemia lymphoma .. .T.B bone marrow
infiltration
bone marrow infiltration
blasts RBCs WBCs

leukoerythroblastic reaction ..... option
bone marrow infiltration leukaemia ,
lymphoma , T.B. , myeloma
leukoerythroblastic reaction
41

clinical pathology
leukaemia

Infiltration bone marrow bone marrow failure
acute leukaemia
immune disorder WBCs antibody auto
immune Infection
tissue infiltration
acute leukaemia
bone marrow failure
aetiology
genetic chemicals ionizing radiation
retrovirus
leukaemia retrovirus
( HTLV1 ( human T-cell lymphotropic virus

49


..............................................................

Acute leukaemia
proliferation blasts
blasts lymphcyte acute lymphoblastic
leukaemia
blasts myelocyte acute myeloid leukaemia


acute lymphoblastic leukaemia
acute myeloid leukaemia young adult
very aggressiveacute myeloid leukaemia

acute lymphoblastic leukaemia ALL %85
common adult


acute myeloid leukaemia very aggressive

acute lymphoblastic leukaemia
lymphoblast
acute myeloid myeloblast promyelocyte
Myelocyte bone marrow
acute myeloid leukaemia N0
to N7 very aggressive leukaemia
leukaemia acute lymphoblastic leukaemia

myeloid
clinical pictures of acute leukaemia
fever infection
sore thorat
pallor fatigue anaemia
Infection neutrophils mature
epistaxis purpuric eruption
fever , anaemia and bleeding
50


..............................................................

Pain bone marrow tissue infiltration
sternum
tender sternum
liver , spleen and lymph nodes enlargement common with
Myeloid lymphoblastic leukaemia
Lymph liver , spleen acute myeloid
nodes
sore thorat , fever , liver , 13
spleen and lymph node enlargement
Infectious mononucleosis ...
acute lymphoblastic leukaemia
child , sore thorat , liver , spleen and lymph node
infectious mononucleosis enlargement
acute lymphoblastic leukaemia
sore thorat infectious mononucleosis

acute leukaemia
thrombocytopenic anaemic
leukaemia

Investigations
total leucocytic count is variable
50,000 WBCs 25%
50,000-5,000 WBCs 50%
WBCs
excessive blast acute leukaemia
in bone marrow
bone marrow blasts
peripheral blood
bone marrow
peripheral blood
51


..............................................................
blasts peripheral blood
blasts acute leukaemia
blasts bone marrow
bone marrow

A leukaemic leukaemia
blasts bone marrow peripheral
blood

How can you suspect Aleukaemic leukaemia
blasts blasts
bone marrow
infection blasts
... bone marrow neutrophils
fever sore thorat
RBCs bone marrow anaemia....
platelets thrombocytopenia
Infection , sore thorat blood
pictures thrombocytopenia
...
normocytic normochromic
hypochromic microcytic ......iron
deficiency anaemia female usually
haemoglobin
normocytic normochromic
Hypochromic microcytic
very mild iron deficiency aneamia
Iron

neutrophils Peripheral blood
52


..............................................................
neutropenia
peripheral blood neutrophils
RBCs Platelets

.I.T.P
thrombocytopenia platelets
bone marrow examination

blasts bone marrow bone


marrow
Aleukaemic leukaemia

examination RBCs platelets bone


..... marrow examination
Auer rods
blasts lymphoblast myeloblast
Auer rods

differential diagnosis
fever with sore thorat acute leukaemia
Infectious mononucleosis
Infectious mononucleosis
poor prognosis
total count 100,000 platelets
L3

general
anaemia
bleeding platelets
Infections antibiotic aerobes and
53


..............................................................
anaerobes Positive negative
acyclovir gancyclovir CMV
sutrim pneumocystis carnii
Lysis
tumor lysis

acute lymphoblastic leukaemia
remission induction
induction of remission
chemotherapy
acute lymphoblastic leukaemia chemotherapy
bone marrow suppression bone marrow blasts
normal blasts peripheral blood


bone marrow malignant
bone marrow malignant
chemotherapy malignant fossi bone
marrow residual normal bone marrow
blasts Peripheral blood

vincristine , adriamycin , predinsolone and L-asparaginase
doses )
(
dose
dose
cardiology
dose doses cardiology
dose vincristine leukaemia
!!
dose haematology

haematology dose
54


..............................................................
Leukaemia


( Remission consolidation (4weeks


cyclophosphamide cytosine arabinoside
consolidation C
C cyclophosphamide cytosine
arabinoside
acute lymphoblastic leukaemia leukaemia
Meninges
prophylaxis CNS CSF methotrexate
cytotoxic drugs CSF

CNS prophylaxis
........
...
CNS prophylaxis
leukaemia


four doses courses text
Haematology


Maintenance therapy
maintenance M m
mercaptopurine and methotrexate

55


..............................................................
acute lymphoblastic leukaemia
vincristine , remission induction
adriamycin, prednisolone and L-asparaginase

signs of remission
clinical picture
% 5 bone marrow blasts
peripheral blood blasts
consolidation consolidation
cytosine cyclophosphamide C C
arabinoside
CSF methotrexate CNS prophylaxis
M M maintenance
methotrexate mercaptopurine

Acute myeloid leukaemia


cytosine remission
cytosine arabinoside Thioguanine-6 arabinoside
ara C ara-c ara-c
Thioguanine-6

Medication consolidation remission

maintenance
bone marrow transplantation remission
very aggressive leukaemia
Maintenance

clinical FAB classification


FAB pathology
L1, classification of acute lymphoblastic leukaemia
56


..............................................................
L2, L3
L1
L2
L3 complication
Prognosis L3
acute myeloid acute
myeloblastic leukaemia myeloblasts
acute lymphoblastic myeloblast
bone marrow ..............
acute myeloid predominant cell
myelobalsts
M0 Undifferentiated
M1 myeloblasts Maturation
M3 promyelocytic DIC
megakaryocytic bone marrow fibrosis
M5 monocytic hypertrphy gum
calssification acute myeloid
M0

immunological
classification
antigens
Myeloid leukaemia antigen

CD cluster differentiation
antigen CD
leukaemias
!!!!
Immune classification cluster of
differentiation antigen Malignant cells
acute myeloid

chronic myeloid leukaemia


57


..............................................................
male middle age massive
splenomegaly
male middle age massive
splenomegaly myeloid

chronic myeloid leukaemia lymphocytic
Lymphnode
spleen
spleen proliferation WBCs
chronic myeloid leukaemia bone marrow
failure late
leukaemia
spleen
bone marrow failure late
WBCs Mature
platelets
leukaemia
chronic myeloid leukaemia
spleen spleen
obese spleen

anaemia bone marrow failure
thrombocytopenia
WBCs mature Infection
anaemia

accidently discovered male
spleen
blood pictures ) - (



function of neutrophils and platelets
Infection bleeding
huge spleen
blast crisis
58


..............................................................
chronic myeloid
acute myeloid Leukaemia


chronic myeloid
blast crisis acute myeloid leukaemia
bone marrow blasts
blast crisis
chronic myeloid chronic myeloid

myeloid line
36 myeloid line myeloblast
promyelocyte myelocyte metamyelocyte
neutrophils
acute myeloid leukaemia
blasts bone marrow
chronic myeloid blasts bone marrow
promyelocyte Myelocyte bone
marrow
spleen .... Myelocyte
promyelocyte peripheral blood
mature neutrophils
platelets
bone marrow failure
suddenly bone marrow
platelets
myeloblast myeloblast
RBCs
chronic myeloid leukaemia
blast crisis
blast crisis accelerated phase
accelerated phase ...
bone marrow Infiltrated anaemic
thrombocytopenic
blast crisis
59


..............................................................
blast crisis acute myeloid
leukaemia
blood pictures
Total leucocytic count 25,000
100,000 chronic myeloid leukaemia
WBCs
acute leukaemia
acute leukaemia 100,000
chronic
It is mainly of myeloid series, in the form
of myelocytes , promyelocytes
Mature neutrophils
Myeloblasts
Myeloblast blast crisis
Basophilia and eosinophilia
leukaemia

investigations
uric acid hyper urcaemia
Philadelphia (ph) chromosome
bone marrow chromosomes and 22 9
Philadelphia chromosome
Philadelphia
Philadelphia chromosome
proliferating programmed cell
death rapidly dividing cells
... bone marrow GIT

tongue
desquamation

folic acid and B12 ... GIT
Bone marrow
60


..............................................................
Philadelphia chromosome

WBCs malignant
bone marrow leukaemia
bone marrow infiltration
leukaemia WBCs
... blast crisis

chronic myeloid
blast crisis
chronic myeloid RBCs platelets
mature
neutrophils
behavior

Leucocytic alkaline phosphatase
is low
chronic myeloid leukaemia

..... ....
WBCs tissue infiltration
leucostasis blood vessel
... Hydroxyurea
Myelosupressive WBCs or 300,000 200,000
20,000 15,000
Leukaemia ...
interferons
Radiotherapy spleen

... leucopharesis malignant


hydroxyurea Myelosupressive
leukaemia
61


..............................................................
... Imatinib
Interferon imatinib
hydroxyurea Hydra
imatinib
blast crisis acute myeloid leukaemia
Imatinib blast crisis
chronic myeloid spleen
WBCs proliferation ...
platelets haemoglobin bone marrow
failure
WBCs functioning
spleen ...

WBCs hydroxyurea
... blast crisis
chronic myeloid
leukaemia Imatinib

chronic lymphocytic leukaemia


Leukaeima ) (
lymphocyte proliferation
... Lymphoid tissue liver, spleen and lymph nodes

bone marrow failure Late

62


..............................................................
chronic myeloid leukaemia
hydroxyurea spleen
blast crisis
chronic lymphocytic lymphocyte
antibody
Liable to infection
Lymphocytes auto antibody
RBCs
Infection
blast crisis
character
cell of origin B-lymphocytes Infiltration
Liver , spleen
immunoglobulins Infection
abnormal immune reaction auto immune
hemolytic
problem
male liver , spleen and lymph node
Persistent lymphocytosis infection
MRSA
stages
Stage A lymph node Lymphocytes
Stage B
Stage C bone marrow failure
Investigations
diagnosis sustained absolute lymphocytosis ....
) ( fever
chest infection antibiotic
0000 ..

investigation feverish ....
CBC
63


..............................................................
CBC Lymphocytosis
... ...
..
..
viral infection
antipyretic
infection
viral lymphocytosis
viral viral bacterial
antipyretic antibiotic viral
...... .. lymphocytosis
chronic lymphocytic leukaemia


bone marrow
failure ... platelets RBCs ..
Infection ... viral bacterial
infection




investigations
Coomb's test
leukaemia auto immune hemolytic


infection antibiotic
bone marrow failure
Stage C bone marrow failure
64


..............................................................
Prednisolone
chemotherapy More aggressive cases

cytoctoxic
C chronic leukaemia chronic C
Chlorambucil cyclophosphamide
episode
...
chronic myeloid leucocytes
.. myeloid myelosuppressive
hydroxyurea
chronic lymphocytic C chronic C
Chlorambucil cyclophosphamide
Lymph nodes
auto immune hemolytic cortisone

Rituximab
Rituximab monoclonal antibody 80-70


... chemotherapy

hairy cell leukaemia


leukaemia MCQ
chronic lymphocytic bone
marrow fibrosis
leukaemia splenomegaly erythema nodosum

Bone marrow fibrosis WBCs cytoplasmic
projection
Interferon therapy
leukaemia interferon
Chronic myeloid leukaemia Interferon
Imatinib
65


..............................................................
Hairy cell leukaemia viral retrovirus
interferon dry tap
dry tap dry tap
needle bone marrow bone marrow

Myelofibrosis
Leukaemia .... Hairy cell
leukaemia interferon

neutropenia and agranulocytosis

neutropenia agranulocytosis

WBCs neutrophils
RBCs normal platelets Normal
aplastic anaemia

viral infection
hypersplenism bone marrow aplasia bone marrow
aplasia neutrophils
hypersplenism neutrophils
Hypersplensim Mono cytopenia
Hypersplenism .... Pan
... mono cytopenia Neutropenia ...
thrombocytopenia

drug phenylbutazone antithyroid

choloramphincol

clinical pictures
sore thorat
sore thorat
ulcers Pus dead polymorph
66


..............................................................
Polymorph Pus
Minimal signs of inflammation Inflammation
organsim neutrophils
. thorat
ulcers Hyperemia signs of
inflammation Minimal scanty pus
blood picture
neutrophils Platelets normal RBCs
Normal
bone marrow examination

antibiotic

blood disease presented with sore thorat and fever

acute leukaemia
acute leukaemia blasts anaemia
thrombocytopenia
blasts bone marrow Peripheral blood

anaemia thrombocytopenia
blood disease
agranulocytosis
neutropenia platelets RBCs
Normal

aplastic anaemia
Pancytopenia
Neutropenia RBCs platelets

agranuloctyosis and aplastic anaemia
67


..............................................................
scanty pus and minimal signs of inflammation
thorat ulcers
minimal signs of throat ulcers
scanty pus inflammation

Myeloproliferative disorders
myeloproliferative
myeloproliferative
WBCs proliferation Leukaemia
RBCs platelets
panhyper cellularity of bone Proliferation
marrow
hyper cellular bone marrow
Hyper cellular fibroblast platelets RBCs WBCs
dominant
polycythaemia rubra vera dominant RBCs
chronic myeloid dominant WBCs
essential dominant platelets
thromobocythemia

polycythemia rubra vera


Pan hyper polycythima rubra vera
bone marrow cellularity
Hyper cellular
RBCs dominant
polycythima rubra .... MCQ
.... platelets .... RBCs vera
WBCs
RBCs

plethora
RBCs Hyperviscosity
68


..............................................................
WBCs Invade tissue
Hyper viscosity
dizziness , vertigo Hyper tension
....Hyperviscosity syndrome
spleen splenomegaly
retina engorged veins fundus examination
itching
Criteria
RBCs ... red cell mass
RBCs packed cell volume
red cell mass
... ...
RBCs ... Red cell mass

haematocrit RBCs mass



labled
dilution
degree of dilution
RBCs
More accurate Haematocrit
haematocrit volume of RBCs total
blood volume
Red cell mass RBCs mass
RBCs blood volume
haematocrit value
Red cell mass More
accurate

secondary polythaemia
hypoxia
69


..............................................................
RBCs .... Hypoxia RBCs
chest disease secondary to hypoxia
splenomegaly
Platelets
criteria B1 platelets 400,000 B2
total leucocytic count 12,000
B3 High leucocytic alkaline phosphatase
WBCs malignant
B4 serum B12
Blood picture RBCs
Bone marrow RBCs dominant
erythropoietin level Low RBCs
erythropoietin !!!
B12 ... B12 ....
chronic myeloid WBCs chronic myeloid
polycythaemia rubra vera WBCs
transcoblamine
B12 chronic myeloid
polycythemia rubra vera
alkaline phosphatase leucocytes

Uric acid


Venesection
Myeloproliferative splenomegaly
basophilia hydroxyurea B12
.
70


..............................................................
hydorxyurea venesection

Allopurinol general support
erythrocytosis Polycythaemia
polycythaemia rubra vera erythrocytosis
COPD hypoxia
secondary COPD
cor pulmonale polycythaemia
... haemoglobin dizziness
....
cushing
polycystic kidney disease polycythaemia
hyper nephroma hydronephrosis
hepatoma

hypoxia secondary
high erythropoietin ....
secondary kidney erythropoietin production
polycythaemia
bone marrow Polycythaemia rubra vera
erythropoietin suppression
itching polycythaemia rubr vera
erythropoientin level to be differentiated splenomegaly
oxygen criteria COPD
Low

Myelofibrosis
proliferation bone marrow elements
bone marrow fibroblast dominant
bone bone marrow fibroblast
fibrous tissue fibrous tissue marrow
megakaryocyte
71


..............................................................
megakaryocytes Malignant malignant
megakaryocytes bone marrow fibroblast
growth factor
fibroblast
bone marrow fibrous tissue
anaemia spleen
extramedullary haematopoiesis
extra medullary haematopoiesis
bone marrow fibrosis bone marrow
stem cells fibroblast
fibrosis stem cells bone marrow
spleen spleen
erythropoiesis
spleen RBCs
extra medullary haematopoiesis
thalassaemia

anaemia splenomegaly
WBCs
chronic myeloid leukaemia anaemia... huge spleen
leucocytosis
differential diagnosis huge spleen


chronic myeloid leukaemia
chronic myeloid leukaemia blood pictures
bone marrow
written Huge spleen
on examination spleen crossing the
umbilicus Huge
myelofibrosis Tear drop cells
dry tap

72


..............................................................
... chronic malaria blood film
tropical splenomegaly.. ..
tropical splenomegaly syndrome

differential diagnosis
Parasite
Kala azar leishmaniesis visceral
leishmaniesis

specific drug for leishmania


amyloidosis spleen

nonhodgkin lymphoma
myelofibrosis
leucoerythroblastic features investigation bone
marrow fibrous tissue
precursor
dry tap trephine from iliac crest
Tear drop cells Investigation

supportive
chapter Hydroxyurea hydra
splenoectomy spleen RBCs
hypersplenism RBCs
bone marrow transplantation
73


..............................................................

Essential thrombocytosis
Platelets 600,000

) ( sustained

thrombocytosis
Platelets Phase reactant rheumatic
fever , rheumatoid Thrombocytosis

essential thrombocytosis sustained
thrombocytosis
hydra hydroxyurea

thrombocytosis reactive
thrombocytosis
splenectomy spleen RBCs

spleen
bleeding hemolysis chronic inflammation
rheumatoid

(plasma cell disorder (Gammopathies



plasma cell disorder
....
...




74


..............................................................

plasma cell
plasma cell Waldenstrom's
) (
Waldenstrom's plasma cell Ig
M
Ig M Waldenstrom's
plasma cell Malignant bone marrow Ig G
or Ig E or IgD multiple myeloma
Multiple myeloma presentation
..... multiple myeloma
Ig G multiple myeloma Ig A
multiple myeloma

Waldenstrom's mono clonal
hyper gamma globulinemia

multiple myeloma Mono clonal
Waldenstrom's mono clonal

Multiple myeloma old male
bony aches ESR

... bone pain plasma
cells cytokines cytokines stimulate
osteoclast osteoporosis verteberal
collapse
vertebral collapse spinal cord
compression
Para plegia
multiple myeloma neuro
Plasma cell blasts bone
marrow ... bone marrow failure anaemia
75


..............................................................
thrombocytopenia
kidney
multiple myeloma kidney
kidney multiple myeloma
Haematological disease diseases
renal multiple myeloma
Leukaemia , lymphoma ...
tumor lysis syndrome
cytotoxic drugs leukaemia , lymphoma
lysis malignant cells
multiple myeloma amyloid
light chain
light chain plasma cell Malignant
bone marrow Gamma globulins
light chain
antibody tubules very toxic
tubules
contrast
Liable nephropathy diabetic
Multiple myeloma light chain reaction
contrast media
multiple myeloma Liable to contrast
nephropathy
infection
bleeding tendency
Plasma cells bone
marrow anaemia thrombocytopenia
acute leukaemia No liver , no splenomegaly
and no lymph nodes

chronic lymphocytic liver ,
spleen and lymph nodes
76


..............................................................
Multiple myeloma Liver spleen lymph
nodes

ESR plasma proteins electrophoresis showing


monoclonal
bone marrow diagnostic .....plsma cells
alkaline phosphatase osteoblast
osteoclast alkaline phosphatase
Normal

light chain light chain
antibody Light chain
heavy chain free light chain Bence
Jones proteins
Bence Jones proteins can not be detected by
deep stick
... albumin

Bence Jones proteins detection Bence
Jones proteins specific
80
clinical pathology

plasma
protein electrophoresis
Normal albumin alpha 1
alpha 2 Beta gamma
abnormality
Gamma
gamma fraction

77


..............................................................
curve
gamma
polyclonal monoclonal

gamma globulin poly clonal
gamma globulin
monoclonal
curve curve
myeloma Staging

supportive
pneumococcal vaccine
Allopurinol gamma globulin

hypercalcemia osteoclast
osteoclast inhibitor
Bisphosphonates
Malignant hyper calcemia
hyper calcemia malignancy
Bisphosphonates
specific therapy VAD vincristine ,
adriamycin and dexamethasone
Thalidomide

Thalidomide the most recent specific treatment
for multiple myeloma
MCQ last minute of MRCP


Waldenstrom's macroglobulinemia

Ig M secreting tumor
78


..............................................................
large molecular weight Ig M
slow in circulation Hyperviscosity
hyper viscosity syndrome fingers cyanosis
hepatosplenomegaly and lymphadenopathy
multiple myeloma
renal disease is not common

hypercalcemia Lesion
IgM
hyperviscosity syndrome

K8 revision
lymphomas
Leukaemia Leukaemia Lymphoma
Infiltration WBCs bone marrow tumor
Lymph nodes spleen liver
bone marrow leukaemia
Liver , spleen and leucocytes Infiltration
lymph nodes
lymphoma
Lymphoid tissue
Peripheral blood malignant cells
Peripheral blood malignant cells
Lymphoid tissue Malignant cells
bone Invade Lymphoma malignant cells
bone marrow invade marrow
(Hodgkin's lymphoma (HL Lymphoma
written lymphoma
lympho adenopathy
... causes of lymph adenopathy
generalized lymphadenopathy
79


..............................................................
liver , spleen and lymph nodes
Leukaemia HIV sarcoidosis Burcella
Infectious mononucleosis
Hodgkin's lymphoma Reed-
sternberg cells
nucleus two kidneys


epidemiology
peak
Hodgkin's
lymphoma Hodgkin's
no relation to Epstein Barr virus
clinical pictures
painless rubbery lymphadenopathy
rubbery consistency lymph
nodes lymphoma rubbery

Liver , spleen and lymph nodes extra
nodal affection is minimal
fever and night sweat
lymphadenopathy .T.B
Lymphadenopathy
Lymphoma
leukaemia
infectious mononucleosis
Brucella
clinincal stages of Hodgkin's lymphoma
Stage 1 one group of lymph
node
Stage 2 two groups lymph node
80


..............................................................
below the diaphragm above diaphragm side

both sides of the lymph nodes stage 3


above and below diaphragm
diffuse stage 4

stage A, B lymphoma
systemic Stage A
systemic systemic Stage B
fever symptoms
investigation
completely CBC full blood count
normal
eosinophilia
... eosinophilia
eosinophilia
High ESR
hyper Lymphoma kidney
kidney Leukaemia urecaemia
definitive lymph node biopsy
management
Lymph nodes Stage 1,2 radiotherapy
pressure symptoms
Stage 3,4 chemotherapy
MOPP chemotherapy
Adriamycin , bleomycin ,) recently ABVD
( vinblastine , Decarbazine
81


..............................................................
recent .. EEACOPP therapy
) Belomycin, Etoposide , Adriamycin, cyclophosphamide ,
( vincristine, procarbazine and prednisone

Non Hodgkin's lymphoma



mediastinal lymph node enlargement
mediastinal lymph node enlargement
sarcoidosis lymphoma Non-
Hodgkin's
.T.B
aetiology relation
Epstein-Barr virus
.... infectious mononucleosis
Long run Non
Hodgkin's lymphoma
Burkitt's lymphoma
Non Hodgkin's Helicobacter pylori

Lymphoid tissue Intestine
Lymphoid tissue MALT
MALT MALT
lymphoma mucosal associated lymphoid tissue
) Helicobacter pylori helicobacter
peptic ulcer incriminated in coronary
heart disease Increminated lymphoma
(
pathogenesis

82


..............................................................
classification
low grade, intermediate grade and high grade

clinical features
Liver , spleen and lymph nodes Hodgkin's
fever .. toxemia
weight loss
systemic manifestations
extra nodal manifestations
Non-Hodgkin's Hodgkin's

investigations
Lymph node biopsy
Investigations
Lymphoma ..... Hodgkin's
hyper calcemia
hyper urecemia investigations Lymphoma
hyper calcemia
Low grade Non-Hodgkin's lymphoma
Rituximab chemotherapy radio therapy
monoclonal antibody therapy
R- CHOP high grade Non-Hodgkin's lymphoma
CHOP
Cyclophophamide,) CHOP
( Doxorubicin, vincristin and prednisolone
(R, means Rituximab ( R-CHOP

Haemolytic anaemias
120 RBC life span
sever Moderate
83


..............................................................
moderate life span of RBCs
sever life span of RBCs

pathophysiology
RBCs bilirubin
bilirubin indirect
Liver Uptake
RBCs .... Liver healthy
uptake ... jaundice
Liver uptake 8
... Hemolysis jaundice
RBCs .... .... .... anaemic
bone marrow replication
replication anaemia sever
Haemolytic anaemia ...hemolysis
jaundice anaemia
compensated hemolytic anaemia
.... RBCs ...
.... RBCs ... blood free haemoglobin
free haemoglobin haemoglobinemia
haptoglobin Hemopexin
haptoglobin hemopexin
.... plasma protein gamma beta
... globulin alpha Beta
alpha and Beta globulins carrier
transferrin thyroxin binding globulins
globulins Liver
Gamma globulins Liver
haptoglobins hemopexins haemoglobin
reticuloendothelial system
Haptoglobin Hemopexin consumed
RBCs .... free haemoglobins ....
haemoglobinuria
84


..............................................................

general features of haemolytic anaemia
pallor ... features of anaemia

Palpitation dizziness fatigue pallor



features of haemolytic jaundice
jaundice .... jaundiced
Indirect bilirubin jaundice
... .... Indirect bilirubin
Acholuric jaundice
haemolytic anaemia Liver ,spleen
site of RBCs destruction spleen
extra medullary haematopoiesis
RBCs spleen , liver
thalassaemia major
myelofibrosis particulary
bone thalassaemia major
bone marrow .. marrow
haematopoesis Liver and spleen
.. bone marrow myelofibrosis
fibrous tissue bone marrow
haemolytic ... leg ulcers
skin Iron .... Iron overload anaemia
skin ulcers
.... hemolysis suggest skin ulcers
sickle cell anaemia
pigment stones gall stones
. pigment stones Hemolytic anaemia
spherocytosis
7 thalassaemia
85


..............................................................
spherocytosis 10 RBCs
spherocytosis pigment ...
pigment load gall bladder pigment stones
MCQ
hemolytic anaemia gall stones
spherocytosis
problem
... jaundiced jaundice
hemolysis Iron overload liver ......hemosidrosis
viral hepatitis gall stone pigmented
intra hepatic biliary dilatation stone
common bile duct
... viral hepatitis viral marker
positive
jaundice
hemolytic crisis
Hemolysis ... hemolysis fever
rigor pallor jaundice ....
bone aches bone marrow Hyper active

hemolytic crisis
aplastic crisis

Hemolysis RBCs
blood ... bone marrow Hyper
active bone marrow Hyper active
bone marrow hyper active
vitamins B12 folic acid
B12 stores
folic acid stores
folic acid deficiency
folic acid
hemolytic anaemia folic acid
B12 iron supplement
86


..............................................................
iron overload
RBCs ... haemoglobin haptoglobin
hemopexin haemoglobin
reticuloendothelial system Organs
Iron overload Iron
overload
bone marrow folic
acid
virus parvovirus
parvovirus ..
erythema face
slapped face
parvovirus bone marrow
bone marrow 3 RBCs
RBCs peripheral blood 3
bone marrow RBCs
Haemolytic anaemia
haemolytic anaemia sever RBCs Life span
20
bone marrow RBCs ...
bone marrow ... hemolysis ...
what is called aplastic crisis
anaemia bone marrow
compensate
hemolytic crisis pallor
haemolytic crisis jaundice
aplastic crisis jaundice
blood pictures
bone marrow hyper active reticulocytosis
aplastic crisis ...nerver , ever reticulocytosis

megaloblastic crisis
folic acid
87


..............................................................


sickle cell anaemia vaso-occlusive crisis

investigatios
haemolytic anaemia blood pictures
Normochromic normocytic anaemia
thalassaemia microcytic hypochromic
folic megaloblastic macrocytic
Hemolysis ... acid deficiency
megalobastic anaemia ...
Hemolysis intra medullary hemolysis

anaemic B12 deficiency


... bilirubin Megaloblastic
cirsis hemolysis manifested
G6PD thalassaemia
leucocytosis , thrombocytosis
bone marrow emeregency hemolysis
hemorrhage hemolysis
thrombocytosis leucocytosis
reticulocytosis Brisk reticulocytosis
hyper active .... hemolysis
hemolysis bone marrow
... hemorrhage reticulocytosis
hemolysis
reticulocytosis impressed
hyper active bone marrow .... hemolysis
hyper active bone marrow hemolysis
88


..............................................................
hemorrhage
bone marrow haemolytic anaemia
hyper active
bone marrow hyper active
normoblast
bone marrow megaloblast folic acid

bone marrow aplasia


MCQ
bone marrow examination of value
Hemolytic anaemia
hyper plasia Normoblast
Megaloblast folic acid
hemolysis bone marrow

haptoglobin haemopexin
consumed
bilirubin

Evidence of intravascular haemolysis
example .... intravascular hemolysis
G6PD
intravascular hemolysis
RBCs .... haemoglobin RBCs
haemoglobinemia free haemoglobin
in peripheral blood
haemoglobin ... peripheral blood
.... haemoglobinuria
..... kidney tubules

Uptake haemoglobin molecules


iron
89


..............................................................
kidney tubules iron
kidney tubules Irritant iron
Iron kidney tubules
urin haemosiderin
kidney tubules ... free haemoglobin
... haemoglobin Uptake ...
) ... tubules haemoglobin
kidney tubules iron ( Iron
haemosiderin
kidney tubules irritate haemosiderin
Urine haemosiderin kidney tubules
haemosidernuria
haemoglobinaemia ... rupture of RBCs...
kidney tubules ..... haemoglobinuria ....
... Haemosiderin haemoglobin
Haemosiderinuria Kidney tubule
intravascular hemolysis Haemosiderinuria
.... iron overload... Haemosiderinuria
haemosidrosis
intravascular hemolysis haemosiderinuria
consumed haemopexin haptoglobin
causes of haemolytic anaemia
Intrinsic and extrinsic
.......
RBCs intrinsic
spherocytosis corpuscular membrane defect
defect in haemoglobin
RBCs ... RBCs extrinsic
auto immune
mechanical hemolysis



90


..............................................................
sites of haemolysis
site of haemolysis extra vascular
reticuloendothelial system spleen
thalassaemia spherocytosis
intravascular RBCs Blood stream
G6PD mechanical haemolysis


hereditary spherocytosis
problem written
membrane defect
membrane defect spectrin
spectrin ... RBCs wall permeable
... .. RBCs spherocyte
wall RBCs
RBCs

Spleen spleen
**** **** )
(

spleen RBCs 120
/ RBCs ATP Na pump 120
. 120 ATP Na pump
...... ..... RBCs
spherical shape trabeculi spleen
phagocytic cells


RBCs biconcave shape

91


..............................................................
/ ) (

RBCs extracellular
) (
* .
*
!!!!!
/ main gates
electron microscope ptn
spectrin neutral ptn
) (ve+ insoluble ptn
.

/ RBCs
/ small gates amino acids
glucose
Main gates
RBCSs
Na pump active pump
ATP
Na pump ATP RBCs
RBCs

spherocytosis

92


..............................................................
gene defect spectrin ptn charge
ve+ .....
main gates RBCs

main gates side gates Na pump
ATP

Na pump
RBCs spherical in shape spleen
phagocytic cells .hemolysis so early......

blood


normal haemoglobin
oxygen RBCs
RBCs spleen

bone marrow bone marrow
proliferating
proliferation

spleen
thalassaemia RBCs
bone marrow
bone marrow
spherocytosis bone marrow RBCs RBCs
spherocyte haemoglobin ...
spleen
mild conservative
93


..............................................................
sever spleen
hereditary spherocytosis features of
anaemia spleen .... .... jaundice
anaemia haemolytic jaundice splenomegaly
family history positive autosomal
dominant
gall stones
Because the bone marrow capacity to
increase erythropoiesis (6 to 8 folds ) exceeds the usual
rate of haemolysis in the disease, anaemia is usually mild
or moderate and may even be absent
RBCs pigment
spleen
thalassaemia

thalassaemia spherocytosis

investigations
anaemic
spherocytosis blood film spherocyte

RBCs ) biconcave shape


( RBCs spherocyte

shperocyte micro spheroctye


RBCs
..... normal RBCs
.... mean corpuscular haemoglobin
concentration high
mean corpuscular haemoglobin
concentration high .... Hyper chromic
...
mean corpuscular volume mean
94


..............................................................
corpuscular haemoglobin concentration
normal .. normal volume
relatively concentrated
osmotic fragility
serial diluation saline
normal saline 0.9
0.9 0.1
normally RBCs 0.4
0.4 RBCs dilution
0.4
RBCs membrane fragile
dilution 0.7 haemolysis
Increased fragility fragility
..... RBCs
... spleen test

10 chromium ....
chromium RBCs

RBCs chromium spleen
..... spleen .... spleen
Gamma camera ...
spleen
RBCs
test Positive spherocytosis
Positive Hyper splenism
hyper splenism
portal hyper tension
.... Hyper splenism
Pan cytopenia
RBCs spleen
chromium RBCs
chromium

95


..............................................................
splenectomy blood transfusion
splenectomy final
not respond family
history
.... spleen spleen
liable pneumococcal and meningococcal
.... infection pneumococcal vaccine
meningeococcal vaccine
recurrence ....
spleens
spleen ... splenic
tissue implantation abdominal wall

...
spleens
recurrence disease
problem
spherocytosis
pallor jaundice .... splenomegaly pallor
...... anaemia jaundice and splenomegaly
haemolytic

.... Positvie family history haemolytic anaemia
positive family history
splenectomy
spleen fever neck
rigidity meningitis
spherocytosis meningitis
Meningitis
original condition
spleen Liable
meningeococcal vaccine pneumococcal
vaccine
Pneumonia
96


..............................................................

Hereditary elliptocytosis
spherocytosis ... mild
hereditary stomatocytosis

(Auto immune haemolytic anaemia (AIHA


haemolytic anaemia adult
auto immune story

45 jaundiced history and examination


.... jaundice and pallor bilirubin Haemoglobin
.... bilirubin ....
.... haemolysis Brisk reticulocytosis ...
anaemic jaundice reticulocytosis
Haemolytic anaemia
45 .... 45 40 ... ....
25 haemolytic anaemia
test coomb's test
haemoglobin electrophoresis
acquired
coomb's test positive Indicate Auto
immune haemolytic anaemia
coomb's test Positive ... negative
antibody cold warm
cold antibody Ig M
warm antibody Ig G

for example
antibody RBCS
antibody RBCS
lupus auto immune haemolytic anaemia
chronic lymphocytic leukaemia
definite diagnosis
97


..............................................................
auto immune haemolytic
... anaemia auto immune haemolytic anaemia
due to wt due to lupus ....due to leukaemia
definite diagnosis
thalassaemia diagnosis sickle cell
diagnosis
shperocytosis diagnosis
auto immune haemolytic anaemia presentation
indicates underlying disease
.... auto immune haemolytic anaemia

auto immune haemolytic anaemia warm


... antibody warm antibody antibody
antibody positive in vitro invitro
active 37 warm
auto immune haemolytic anaemia warm
antibody
L ....leukaemia , lymphoma lupus

alpha methyl dopa
manifestations of haemolytic anaemia
jaundice .... Pallor anaemia
spleen Palpable
Investigations
coomb's test antibody Ig G

blood spheroctye

Ig G RBCs ... spherocytes Ig
G RBCs spherocyte
acquired spherocytosis
spherocytosis Lupus auto
immune
Leukaemia .. acquired
spherocytosis lupus
98


..............................................................
Leukaemia acquired
) ( Evan's syndrome antibody
platelets
.... Problem problem
Evan's syndrome
Investigations
..... cause investigations
coomb's test Ig G .... warm
Lupus .... Leukaemia Lupus marker

cortisone
splenectomy ...
.... spherocytosis .... spleen
spleen .... extravascular blood stream

auto immune haemolytic anaemia


cold antibody
antibody cold active ... 37
..... in vitro .. cold .... viral
infection infectious mononucleosis

Infectious mononucleosis
sore thorat ..... jaundice
jaundice auto immune
haemolytic anaemia Epstein Barr virus
hepatitis
coomb's test
transaminases
fever and sore thorat
Infectious mononucleosis
99


..............................................................
..... jaundice jaundice auto
immune haemolytic coomb's test
positive
Epstein Barr virus viral hepatitis

anti body Ig M
warm antibody Ig G
cold anti body Ig M
Ig M ... M
MCQ

steroid
cold antibody intravascular
extravascular

paroxysmal cold haemoglobinuria


.... ....
....... haemolysis measeles
mumps chicken pox
coomb's test ... coomb's
test test to detect anti body RBCs

.. direct coomb's test detect anti body...
..... RBCs RBCs
..... auto immune
anti body RBCs
RBCs
direct coomb's test detect antibody coating
RBCs
100


..............................................................
RBCs human serum
......

in direct coomb's test


RBCs .... Plasma
antibody
anti body ... RBCs
..... RBCs

anti body RBCs RBCs
plasma
coomb's test direct indirect
..


Drugs causing haemolysis
alpha methyl dopa
Hapten penicillins cephalosporins
incomplete antigen .... .... RBCs
RBCs .. complete antigen
Quinidine type
innocent by stander innocent by
stander
sulpha sulpha
..... albumin ..... sulpha
albumin .... antigen ..... antibody
complement ....
..... sulpha sulpha ..... albumin
..... antigen antigen ..... antibody
complement circulation
RBCs
!!!! Innocent by stander........
101


..............................................................
antigen antibody complement
... complex ....
... .... RBCs
complex ...
mechanism ....

antibody ... RBCs
albumin trigger Immune system

Haemolysis
G6PD antimalarial

drugs causing jaundice
hepatotoxic drugs liver cell
failure cholestasis ...
haemolysis fungal
alpha methyl dopa Hapten mechanism
sulpha
innocent by stander

mechanical haemolysis
RBCs mechanically
haemolysis .....
... sole capillaries ...
RBCs sole
prolonged marching
.... ...
... .... ....
...


prosthetic valve
102


..............................................................
... ... problem
.... double mitral ....
... valve replacement ..... endocarditis
haemolytic anaemia mechanically
calcified aortic valve Haemolysis
Micro-angio-pathic haemolytic anaemia
micro-angio-pathic haemolytic anaemia micro
angio ..... blood vessel ........pathic
disease

blood vessel fibrin thrombus
fibrin thrombus
..... fibrin thrombus

RBCs
...... fibrin thrombus
mechanical haemolysis
fibrin thrombus ............ DIC
haemolytic uraemic syndrome TTP
................ thrombotic thrombocytopenic purpura
scleroderma scelroderma
fibrous tissue collagen deposition wall blood
....... vessels malignant hypertension
MCQ RBCs shistocytes
fragmented RBCs helmet
DIC haemolysis jaundice
Shistocytes
shistocytes blood film

toxic causes of haemolysis


103


..............................................................
malaria .......... ...... clostridium
snake venom .....

...
...


...... .... .....

(paroxysmal nocturnal haemglobinuria (PNH


...... RBCs


acquired defect .... stem cell stem cells
...... mother cells stem cells
RBCs abnormal platelets
abnormal WBCs abnormal
abnormality in RBCs RBCs Specific protein
cell membrane glycosoyl phosphatidyl
(inositol (GPI
...... ...... ..... RBCs
........ RBCs )(GPI
..... complement .........
) (
Infection
complement PH blood
acidic side
..... stagnation of circulation

..... CO2 retention circulation ....
PH low PH
excite complement
complement RBCs
) (GPI complement
104


..............................................................
RBCs
RBCs .....
........ complement ....
RBCs

complement no antibody......
Infection

...

platelets aggregation..... ......


WBCs ....
haemolysis recurrent vascular
occlusion Infection
sickle cell
haemolysis vascular occlusion Infection
sickle cell Infection auto
splenectomy spleen

Venous thrombotic episodes
.... ..... hepatic veins
Budd chiari syndrome
problem
..... anaemic ...... ...... Infection
..... vascular occlusion Budd chiari

Investigations

test ( Ham's test (old test
..... acidic media ....
105


..............................................................

flow cytometry
leukopenia thrombocytopenia
....
haemolytic anaemia acquired
..... virus
stem cells
it may lead to aplastic anaemia
.... haemolytic anaemia
aplastic anemia ...... bone
marrow aplasia haemolytic anaemia
parvo virus transient
haemolytic anemia aplastic
........ anaemia paroxysmal nocturnal
haemoglobinuria
Investigation
coomb's test negative
Intravascular
G6PD

steroid

Thalassaemia
Thalass
aemia

Beta thalassaemia
106


..............................................................

( homozygous (Thalassaemia major

heterozygous ( Thalassaemia minor) i.e. trait
thalassaemia minor mild anaemia
microcytic hypochromic
iron deficiency anaemia
splenomegaly F ....
hypochromic
.... microcytic .... iron Iron
........... ..... iron
thalassaemia minor Iron deficiency
resistance
haemoglobin electrophoresis
... .... female Iron
deficiency commonest cause of anaemia
iron
electrophoresis thalassaemia minor
diagnosis of thalassaemia minor
family consuling
thalassaemia minor
thalassaemia minor written ...........
thalassaemia minor thalassaemia major
thalassaemia minor .... differential
diagnosis ...... iron deficiency
........ sidroblastic anaemia ........

thalassaemia minor clinically


sidroblastic iron deficiency splenomegaly
Investigations ... electrophoresis
... thalassaemia minor
.... Minor ... 10
thalassaemia minor
107


..............................................................
..... thalassaemia minor thalassaemia major
RBCs

thalassaemia intermedia
..... .....

it includes patients who are symptomatic with moderate
anaemia
7 10
it is more sever than thalassaemia trait
thalassaemia trait thalassaemia minor
thalassaemia intermedia milder than
thalassaemia major
minor major
thalassaemia intermedia
age of presentation ... later in age....
late childhood 15
..... thalassaemia thalassaemia minor
..... thalassaemia major major
Presented thalassaemia major Presented
early childhood

thalassaemia major

anaemia iron over load
.... sidroblastic anaemia Iron Iron
protoporphyrin iron .....deposition
thalassaemia major aplastic anaemia
anaemia with iron over load
anaemias
tissue iron .... serum ferrittin
transferrin saturation
108


..............................................................
.... haemoglobin alpha beta chain
.... alpha gamma
... ..... switch gamma
beta
Normal haemoglobin ..... alpha
beta
..... alpha gamma
gamma switch beta
alpha .... gamma
switch beta
..... haemoglobin F alpha
gamma ... fetal haemoglobin
haemoglobin F

.....
thalassaemia Beta chain
alpha chain beta chain
alpha ...... beta gamma
alpha ..... gamma haemoglobin F
alpha chain .... gamma
.... gamma alpha chain
free alpha chain
free alpha chain RBCs
bone marrow .... haemoglobin F
Haemoglobin F .... alpha chains gamma
alpha chains
Beta chain
beta
free alpha chain life span of RBCs
RBCs ...... spleen Haemolysis
spleen spherocytosis

bone marrow Hyperactive haemolytic
RBCs Free alpha chain
109


..............................................................
Free alpha chain bone marrow
bone marrow ...
RBCs ..... free
alpha chain
RBCs free alpha chain
RBCs bone marrow
RBCs bone marrow peripheral
blood ...... bone marrow peripheral
blood spleen
free alpha chain .. chemical

RBCs spleen ....
bone marrow Hyper active spherocytosis
spherocytosis bone marrow
thalassaemia bone marrow

ineffective erythropioesis
thalassaeima spherocytosis
23

free alpha chains RBCs spleen bone
marrow
thalassaemia bone marrow ....ineffective
.... erythropoiesis bone marrow
extra medullary haematopioesis
liver spleen RBCs

clinical pictures
6
Pictures of haemolytic anaemia
pallor jaundice splenomegaly
.... Mongoloid features mongoloid features
thalassaemia sperocytosis
110


..............................................................
iron over load thalassaemia
...
Pale jaundice Iron over load skin
Iron overload skin Hyper pigmentation

...
skin
by inspection spherocytosis
thalassaemia
color of the
skin
Mongoloid features

investigations
....... anaemia
haemolysis High indirect bilirubin
reticulocytosis
blood film target cells
... haemoglobin electrophoresis haemoglobin F
X-ray skull ....hair on end appearance
long bones .....showing thin cortex & wide medulla
mean corpuscular volume .... microcytic
.....hypochromic normochomic
high serum ferritin iron over load
featus
biopsy placenta
.....chorionic villus biopsy
thalassaemia....

blood transfusion
folic acid bone marrow hyper active
111


..............................................................
desferal
bone marrow transplantation
spherocytosis ...... spherocytosis bone marrow
RBCs .... .... ... spleen
spleen bone marrow
thalassaemia bone marrow ....
bone marrow transplantation
Indication splenectomy
Indication of splenectomy
..... hypersplenism hypersplenism blood
transfusion requirements 250
packed RBCs/Kg/year

30 ........
....... blood transfusion .... dose
spleen Hypersplenism
spleen extra medullary
haematopoiesis .... haemopoesis
... hypersplenism spleen .... die
bone marrow transplantation

sickle cell anaemia


haemoglobin S
haemoglobin F adult haemogobin
haemoglobin F Oxygen delivery
... ...
...
haemoglobin ... adult
haemoglobin S .... delivery


..... hypoxia sickling

112


..............................................................
sickle cell anaemia sickle cell trait
sickle cell anaemia abnormal genes 2
sickle cell heterozygous one abnormal gene
trait
Manifestations sickle cell anaemia
anaesthesia hypoxia sickle cell trait


Just .... haemoglobin A haemoglobin S
one amino acid
valine glutamic
haemoglobin S
oxygen delivery ...
hypoxia haemoglobin S RBCs
sickling
damage .... macro infraction sickle
small blood vessels .....painful micro infraction
crises
Haemolysis features of anaemia
without splenomegaly
haemolytic anaemia spleen

haemolytic anaemia without splenomegaly
spleen sickle cell anaemia
autosplenectomy repeated splenic infarction

intra vascular haemolysis G6PD
..... spleen

113


..............................................................
paroxysmal nocturnal haemoglobinuria
complement

auto immune cold


mechanical haemolytic anaemia
intravascular haemolysis
haemoglobinuria haemosiderinuria...

extra vascular
spherocytosis thalassaemia
extra vascular blood stream
vascular occlusion
High mortality

Dactylitis
Mesenteric occlusion
infraction nephrogenic diabetes insipidus

sicklin renal medulla

sickling RBCs
platelets
blood vessel ischaemia
sickling renal medulla. Less
vascular ...... cortex ...... hyper tonic
renal medulla ..... sickling
sickling renal medulla ischaemia renal
medulla
tubules renal medulla loop of
114


..............................................................
Henle .... collecting tubules anti diuretic
hormone
Nephrogenic diabetes insipidus
sickling ..... renal medulla
..... ischaemia Hyper tonia medulla
ischaemia renal tubules medulla
..... loop of Henle and collecting tubules
diabetes insipidus

.... ...
sickle cell ... sickle
cell diabetes
sickle cell diabetes ....
sickling ... sickle

diabetes insipidus diabetes mellitus


vascular occlusion children

sickle cell anaemia
vasculitis Henoch Schonlein purpura
vasculitis far east Kawasaki

myocardial infraction
.... coronary care unit
.... !!!
coronary Kawasaki sickle cell
myocardial infraction in child
myocardial infraction
vascular occlusion
retina blind
blood vessel
115


..............................................................
long term sequalae
Hyposplenism ... Infection
auto splenectomy Hyposplenism
salmonella pneumococci infection
... meningeo

we can summarize the clinical manifestations of sickle cell
anaemia
-manifestations related to the haemolysis 1

manifestations related to the vaso occlusion painful -2
crises and organ failure
manifestations related to infections with pneumococci -3
and salmonella

General investigations
High indirect bilirubin ...... haemolysis
reticulocytosis
Haemoglobin S haemoglobin electrophoresis
High
sickling test
Na slide RBC
.......... hypoxia Na bisulphate ...... bisulphate
sickling.............. slide Hypoxia RBCs
slide
sickle cell trait sickle cell anaemia Positive

haemoglobin S very
sickle cell anaemia high
sickle cell trait very high haemoglobin S
116


..............................................................

sickle cell beta thalassaemia

anaemic High level of haemoglobin S
... splenomegaly problems

sickle cell anaemia spleen


spleen sickle cell Beta
thalassaemia

sickle cell anaemia ...... vascular
occlusion sickling
sickle cell beta thalassaemia ....
haemoglobin S haemoglobin F RBCs
haemoglobin F sickling
haemoglobin S.... hypoxia RBCs
sickling
haemoglobin S Haemoglobin F sickling
Haemoglobin S
haemoglobin F RBCs
oxygen haemoglobin
...... F
haemoglobin S hypoxia sickling RBCs

haemoglobin S .... haemoglobin F



..... Hypoxia sickling RBCs
....... Haemoglobin F
.... haemoglobin S Oxygen
hypoxia .... oxygen
RBCs haemoglobin F haemoglobin S
.... .... haemoglobin S
Hypoxia haemoglobin oxygen
117


..............................................................
Hypoxia haemoglobin F
haemoglobin S sickling

sickle beta thalassaemia Life span
sickling
auto splenoectomy
spleen
sickle cell anaemia
spleen ...... sickling ..... splenic vein
splenic vein spleen ... congested

blood transfusion
(sickling crises (pain
analgesics ..... Na HCO3
Na HCO3 ..... lactic acidosis vascular
occlusion ischaemia Lactic acidosis
fresh blood fresh ...........
oxygen

.. haemoglobin S haemoglobin F
... Haemoglobin F applied
cytotoxic drugs haemoglobin F

pneumococal influenza vaccines


bone marrow transplantation

118


..............................................................
Haemolytic anaemia
Gluose 6 phosphate dehydrogenase (G6PD) deficiency
thalassaemia spherocytosis G6PD
problems sickle cells problems
clinical pathology blood
hexose monophosphate shunt
NADPH H
H glutathione reduced RBCs
RBCs glutathione
glutathione reduced RBCs
oxidizing agent
glutathione reduced H
....... NADPH NADPH hexose
monophosphate shunt
shunt G6PD
..... G6PD hexose monophosphate
shunt .... NADPH ........ H glutathione
reduced RBCs
RBCs
oxidizing agent RBCs

.... very mild disease

oxidizing agent

chronic haemolysis aggrevating
oxidizing agent
favism
favism )
(
favism D6PD ..... abnormal
metabolism ... beans
119


..............................................................

oxidizing agent
G6PD deficiency
favism ... favism ..... two abnormalities
G6PD oxidizing
clinical presentations syndromes of G6PD
deficiency
Acute drug induced haemolysis -1
antimalarial sulpha
haemolysis Intravascular
no splenomegaly
chronic haemolytic anemia -2
favism-3
neonatal jaundice-4
infections and acute illness will precipitate haemolysis -5


favism .... ...
haemolysis
haemolysis self limited self
limited
haemolysis
bone marrow hyperactivity
....... reticulocytosis reticulocytes rich

.. Haemolysis ......
emergency Haemolysis acute
12

... reticulocytosis
reticulocytes very rich ..... G6PD
adult RBCs
120


..............................................................
Normally adult RBCs
G6PD ..... reticulocytes


... . bone
marrow reticulocytes ....

assasment enzymes
enzyme deficient
Haemolysis
G6PD adult mature RBCs
attack immediately attack
reticulocytosis G6PD normal or high ..

assessment

haemolytic crises
.... reticulocytosis blood
.... mature
pictures RBCs
enzyme Mature RBCs

Hienz bodies
Hienz bodies )
(

haemolysis intravascular blood film


Hienz bodies
oxidized haemoglobin RBCs
haemoglobin RBCs

Hienz bodies
121


..............................................................

Avoid cause
blood transfusion
splenectomy

anaemia


reduction below normal below what is expected

.... .... below what is expected


...... Normal normal haemoglobin
100 100
reduction below what is expected in one of the following
parameter
parameter expected anaemia
percentage RBCs count
haematocrit
parameter
.... .... normal normal
... ...

what is expected
definition
age sex

intrauterine relatively hypoxic environment
122


..............................................................


Hypoxia main stimulant for RBCs
formation

relatively hypoxic environment
) (
... normal polycythaemia

19 ... Normal 14
... ) (


18 ........ ...
stimulus ... hypoxia
18 .... ............ 9


....
9
Normal
adult
15-14 Puberity 15
15


72 15

N.B. reduction of haemoglobin in old age is adisease

15

18 9 9 ...... 15
123


..............................................................


K9 revision

Haemostasis and bleeding disorders
haemostasis stopping of bleeding prevention of
blood loss
bleeding
....

vasoconstriction
platelet plug
blood vessel vaso spasm
blood vessel blood vessel Platelet
plug
formation of blood clot coagulation
repair damaged blood vessel
Haemostasis

coagulation factors platelets blood vessel wall
.. blood vessel
wall
platelets
coagulation
platelets
150,000 400,000
thrombocytopenia 150,000 significant
thrombocytopenia 100,000
Platelets 150,000
clinical haemorrhage platelets
124


..............................................................

Platelets
10,000 20,000 .... 10,000
Platelets

10,000 cerebral haemorrhage
rate of drop of platelet count is very important

liver
cirrhosis Hyper splenism Platelets 40,000
.... rate of drop of platelets very
gradual
thrombocytopenia
platelets ...... Normal ..... 20,000

platelet count 80,000 to 100,000 is adequate for
haemostasis

blood pictures normal

platelets 70,000
..... anti
coagulant component

anticoagulant
platelets
consumption

...

splenectomy .... platelets
Hypersplenism
Hypersplenism Liver cirrhosis portal
125


..............................................................
hypertension splenectomy
bleeding time

ear lobule
neruse
filter paper

.......
filter paper 00008
bleeding time


Platelets
....
blood clot
blood clot platelets
plug

Ivy's method Duke's method ...

bleeding time :
quantitive defect in platelets platelets
Platelets normal thromboasthenia
platelets dysfunction uraemia
vascular defect vasculitis Henoch
Scholein purpura

Platelets functions

thromboasthenia platelets
count Normal bleeding time prolonged
mostly in platelets vascular

126


..............................................................
platelets functions
platelets adhesion platelets
wall blood vessel
blood vessel wall ..... .....
Platelets wall blood vessel
..... platelets adhesion
platelets aggregation
Platelets
) (

....... adhesion
aggregation
aggregation ADP
ristocetin
platelets aggregation ristocetin

test
Hess capillary fragility test
Hess test .... test

capillary fragility
Henoch Scholein purpura


systolic .... diastolic
systolic diastolic ..... capillary pressure
... ... forearm
Petichae
test ......no comment


127


..............................................................
vasculitis
.... vasculitis platelets
bleeding time Normal
... )
( Henoch Scholein purpura
vascular occlusion
blood vessel ....
.... bleeding time may be normal
) (
Role of platelets in haemostasis
written
5 written
haemostasis
... clinical pathology


59
blood vessel
.... ......... trauma ) exposure
blood vessel endothelium
endothelium (.. collagen fibers blood
vessel ..... endothelium ..... collagen fibers

Injury release..... VWF


VWF ... adhesion............ platelets
adhesion
VWF disease adhesion defect
platelets adhesion platelets
release
platelets prostaglandins ADP
stimulation platelets aggregation
platelets
platelets aggregation platelets plug

128


..............................................................

trauma
vasoconstriction ) nervous reflex
( myogenic contraction release of
Thromboxyane A2 serotonin
platelets
vasoconstriction
Thromboxyane A2 vasoconstriction
enhance platelet aggregation

trauma release tissue thrombo-plastin
enhance coagulation
coagulation fibrin


platelets adhesion platelets aggregation

vasoconstriction

coagulation

129


..............................................................

..... vasoconstriction platelets


.......... coagulation
sequances vasoconstriction Platelets
adhesion platelets plug coagulation
fibrin platelets plug
Platelets plug ...
.... cement substance
fibrin coagulation
platelet release Platelets
enhance coagulation

other function of platelets


platelets function growth
130


..............................................................
factors multiplication growth of fibroblast
...
blood vessel wall platelets growth
factors collagen wall
blood vessel

repair wall

vaso constriction platelet plug coagulation
... blood vessel .... collagen
... platelets growth factors

Process of coagulation
coagulation coagulation
....
Intrinsic pathway extrinsic pathway
extrinsic pathway

coagulation fibrin
extrinsic .......extrinsic
trauama
coagulation tissue thromboplastin ....
tissue thromboplastin activation factor
common pathway
.... vasoconstriction ....platelet
adhesion .....platelet plug ....tissue thromboplastin
..... ....factor 7 .... common pathway ..... fibrin
platelets
.... platelets growth factor blood vessel

Intrinsic pathway
131


..............................................................
Intrinsic
.... .... coronary
.... DVT .... intrinsic
pathway
rough surface
atheroma atherosclerosis
atheroma rough surface
Intrinsic pathway
extrinsic pathway extrinsic emergency
intrinsic ... extrinsic
Protection
Intrinsic

endothelium
atheroma activation
factor 12

factor 12 activation activation factor 11
factor 11 active activation of factor 9
factor 9 activation active 8
common pathway
extrinsic Intrinsic
common path way

sequances
common pathway
Intrinsic extrinsic
factor 10
factor 10 prothrombin thrombin
thrombin fibrinogen fibrin
common pathway
fibrin stabilization
132


..............................................................
factor 13 fibrin stabilization factor

extrinsic pathway
... tissue ...... tissue thromboplastin
tissue thromboplastin .... 7 factor
common pathway
Intrinsic pathway
rough surface
...... factor 12 factor 11 ....active
..... factor 11 ..... 9 9 factor 8
factor 10 .... Intrinsic extrinsic
factor
factor Intrinsic extrinsic
common pathway prothrombin thrombin
fibrinogen fibrin

trauma extrinsic pathway
intrinsic :
) ( intrinsic
pathway
trauma extrinsic intrinsic

coagulation profile
Prothrombin time partial thromboplastin time PPT
Prothrombin time extrinsic pathway PPT
Intrinsic pathway
platelets ...
bleeding time defect
Platelets ... platelets count normal
bleeding time ..... Platelets dysfunction
133


..............................................................
dysfunction adhesion aggregation
platelets function test ....
platelets ....... .... defect in coagulation
extrinsic pathway ....... prothrombin time Intrinsic
pathway PPT .....
coagulation profile
... written bleeding tendency

...
platelets count
bleeding time platelet functions PT PPT

factors of coagulatios



fibrinogen
prothrombin
prothrombin
thrombopalstin
calcium
proaccelerin and proconvertin
!!!
anti haemophilic globulin
Christmas
Stuart prower factor
Hegman factor Hegman
factor 8 factor 8 C
caogulation
factor 8VW platelets adhesion
factor 8 Ag
anti body factor 8

134


..............................................................
intrinsic pathway
intrinsic pathway
factor 12 Hegman
11 9 common pathway
factor 12 Hegman active
....
Invitro
test tube


!!!! blood vessel wall
Intrinsic pathway
activation of Hegman factor
12 11 9 8 common
pathway
blood clot
clotting time


....... fibrin

test tube incubator
clotting time
Whole blood clotting time
5 10 ....

... clotting time 10 -5
..
... activation of Hegman factor
activate
..........
Hegman factor
intrinsic common pathway .... 30

135


..............................................................

clotting time
Hegman factor defect factor factor
factor Hegman
factor 30 40

range clotting time


Insensitive
Hegman factor
Hegman factor
activation
activation factor 12
pathway 30 35

factors 35-30
factor defect


... ... range



30
PPT
PPT
intrinsic pathway
clotting time accurate
PPT
haemophilia
haemophilia very mild clotting time
Normal
PPT More accurate

heparin ....affects clotting time & PPT

136


..............................................................
Intrinisc pathway
... PPT prolonged

PPT
PPT .... double
anti coagulation
test extrinsic pathway

tissue thromboplastin factor 7
Pathway ....prothrombin time
extrinsic pathway

thromboplastin
extrinsic trauma thrombopalstin
thromboplastin extrinsic pathway
15
prothrombin time
factor 7 factors Pathway
factors highly related vitamin k dependant
prothrombin
extrinsic pathway factors
vitamin K dependant factor
prothrombin group

prothrombin time
test extrinsic pathway
......... ....
extrinsic pathway .... Prothrombin time
liver function test
prothrombin dependant
liver Prothrombin time
prothrombin time
137


..............................................................
prolonged
oral anti coagulant
Oral anti coagulant anti vitamin K
coagulation factor
pathway
oral anti coagulant prothrombin time
double
triple oral anti coagulant
Prothrombin time liver function test


prothrombin time .... 14
.. control ...
.... .... ideal
...
test
prothrombin time 14
control 14
14
16 prolonged....
20
control patient
ratio

14
14
ratio ... 14/14
...

control 14
28
28 14 2
bleeding tendency

138


..............................................................
INR International normalized ratio
equation ratio PT
PT factor
INR ...
INR ... 0.8

INR .... 1.2

INR 0.8 1.2


INR more and more bleeding
tendency
oral anti coagulant INR

INR 3-2

oral anti coagulant INR



INR 3-2


INR 2.5 ........ 3.5 2.5 3.5
....... ... 2.5

... ..

liver INR ....
....

139


..............................................................
0.8
3.. 2.... 1.5...
INR Liver
Oral ...
liver ...
bilirubin albumin INR
liver

fibrinolytic system
plasminogen
tissue plasminogen activator plasmin
plasmin fibrin FDP )Fibrin degradation
( product
fibrinolytic system
Inhibitors of coagulation

anti thrombin 3 protein C protein S
protein C thrombin-thrombomodulin
complex
acitive protein C inactivates factor 5 and factor
8a inactivation enhanced by protein S

natural anti coagulant
protein C and S anti thrombin 3
vitamin K liver factors of
coagulation vitamin K dependant
prothrombin
factor 2 ,7
vitamin K Liver anti
coagulant
protein C and S .... anti thrombin
140


..............................................................

clinical MCQ written


pathology

purpura
blood
Purpura

Platelets disorder
thrombocytopenia ....... thrombocytopenia
ITP platelets survival ....
auto immune hypersplensim
.... thrombocytopenia
vit. B12 ... decrease of the platelets production
folic acid deficiency
bone marrow bone marrow depression
infiltration
uraemia
congenital deficiency of megakaryoctes CFUs
drugs

increase of .... thrombocytopenia


consumption
increase of platelets consumption....
TTP DIC
alcohol , thiazides , estrogens and
megakaryocytic Inhibition cyclophosphamide
series production
penicillin , cephalosporines, sulfa and methyl
Platelets ... dopa
141


..............................................................
destruction through immune mechanism

may lead to thrombocytopenia
Platelets
.. thrombocytopenia
thrombocytopenia

Purpura platelets disorder



thromboathenia
acquired
Von willebrand's disease
((VWD ) (
acquired drugs uraemia

purpura Platelets disorder



thrombocytosis
thrombocytosis Purpura
... Platelets dysfunction

Purpura
vascular purpura
.... senile purpura
blood vessel subcutaneous ...
blood vessel collagen fibers blood
vessel
...... perivascular collagen fibers
.... ... ..
brownish
142


..............................................................
Haemosiderin ....
...
...
senile purpura
... ... ... Purpura

... benign condition
allergic purpura
Henoch Schonlein purpura
Infection
meningitis Meningeo coccal
meningitis
... ...
neck rigidity meningitis Purpuric
eruption
Neck rigidity meningitis
purpura purpura fulminans
sever infections
purpura simplex
Non specific
...


... coagulation
.... profile PT extrinsic pathway PTT
Intrinsic pathway
platelets count bleeding time
Normal ...
...
Non steroidal anti inflammatory durgs
vitamins vitamin C
collagen wall blood vessel

143


..............................................................
clinical picture
purpura
.... skin multiple petichael
haemorrhage without raised edge in platelets disorder
raised edges vasculitis
bleeding per orifices and mucous membranes
Purpura
.. .... female ....vaginal bleeding
Purpura
Haemophilia
Haematoma, ecchymosis, haemarthrosis

Platelets defect Purpura common
spontaneous post traumatic bleeding


disorders of platelets
Idiopathic thrombocytopenic purpura-1

auto immune
platelets ..... antibody antibody
platelets reticuloendothelial system
Ig G
platelets .... .... spleen
... spleen .... splenic receptors spleen

.... reticuloendothelial system
..... cortisone spleen

...
Purpura sudden onset
144


..............................................................
adult ... Purpura gradual onset
platelets 40,000
adult ... Platelets
haemolytic anaemia
thrombocytopenia Evan's syndrome
clinical pictures
purpuric eruption ... MCQ
idiopathic thrombocytopenic purpura
splenomegaly
MCQ common
splenomegaly ....Liver cirrhosis ..... leukaemia
...... Infectious mononucleosis .... Infective
endocarditis .... common ITP
..... splenomegaly
Investigation
low platelets count
by exclusion
... ...
.... Purpuric eruption
.... blood picture Platelets ...
RBCs WBCs
Platelets
.... Pancytopenia aplastic anaemia
Hypersplenism ... Platelets
.....
...
spleen spleen thrombocytopenia ...

by exclusion ITP

.... bone marrow .
to exclude similar condition
Leukaemia bone marrow infiltration
145


..............................................................
rare thrombocytopenia
bone marrow examination
ITP

megakaryocytes
... Hyper plasia
budding budding
budding sign Megakaryocytes
ITP ...


.... ..
symptomatizing Platelets
..
.. ...
.... ...

Platelets .... Platelets


... .......... .....

) (
platelets 48 10,000
Platelets

.... for long time
.. Platelets 3
platelets count .... 12 ...
..

"


48 ...
146


..............................................................
..
...
bone marrow investigations
...
.... ITP
adult
adult ... ...
adult
splenectomy
splenectomy
..... splenectomy Immune suppressive
immune suppressive
spleen spleen



platelets 30,000
30,000 .... 20,000 ......
.... blood
transfusion
RBCs ... Platelets WBCs
dilulation thrombocytopenia
..... whole blood platelets concentrate
.... .. ...
.. ..
...

...
... ...

platelets concentrate

147


..............................................................

Blocking Anti body
anti body ... receptors
spleen
... anti body antibody receptors
... spleen platelets splenic receptor

platelets consumption syndrome
MCQ
DIC , TTP , haemolytic uraemic syndrome
( TTP (thrombotic thrombocytopenic purpura
female thrombocytopenia
thrombic thrombocytopenic purpura

consume ... platelets thrombocytopenia
... thrombotic kidney
TTP

... ..

haemolytic uraemic syndrome
Haemolysis and renal failure

haemolytic uraemic syndrome
following gastro enteritis
E-coli
Ecoli
Oral
pregnancy
148


..............................................................
... systemic
.... following attack of diarrhea attack of
diarrhea followed by systemic disease

) haemolytic uraemic syndrome
TTP
haemolytic uraemic syndrome
.... .....
consumption .... Platelets
.... blood vessels RBCs
micro angiopathic haemolytic anaemia
DIC dissemenated
DIC disseminated
TTP
haemolytic uraemic syndrome (
Reiter's syndrome
dysentery
....
... Reiter's syndrome
gastroenteritis renal
haemolysis ... Haemolytic uraemic
syndrome

qualitative platelet Disorder
Inherited adhesion defect aggregation
defect
inherited aggregation defect Glanzmann's
syndrome
inherited adhesion defect Bernard soulier
syndrome Von Willebrand's disease
149


..............................................................
Von Willerbarnd's disease
Von Willerbrand factor
Liver ..... .... endothelium factor 8
Liver ... factor 8
Liver
anti haemophilic factor liver
Von Willebrand factor endothelium
..
Von Willebrand's disease

it is the most common inherited bleeding disorder
Haemophilia ...
......... mainly autosomal dominant
mainly autosomal dominant
Von Willebrand factor
adhesion
adhesion defect

Platelets count normal

investigation VWD
Prolonged bleeding time + normal
platelets count

platelets dysfunction
platelets aggregation normal platelets
adhesion abnormal
...
Von Willebrand factor ..

150


..............................................................
.... blood vessel wall
Platelets
Platelets
adhesion defect .... adhesion

coagulation Von Willebrand disease
coagulation defect some time defect
carrier Von Willebrand factor
factor 8
anti haemophilic factor factor
anti haemophilic carrier Von Willebrand factor
factor
anti haemophilic factor ..... Von Willebrand factor


Haemophilia
coagulation defect
mainly Von Willebrand
autosomal dominant
... adhesion Von Willebrand factor
normal Platelet count .. bleeding
platelet aggregation ... platelets function
platelet adhesion ...

type1, type2 and type3

factor 8 concentrate
fresh frozen plasma cryoprecipitate

vaso anti diuretic hormone ....
pressin
blood vessel
ADH
release Von vaso constrictor vaso pressin
151


..............................................................
endothelium Willebrand factor

DDAVP (synthetic ADH) release of VWF


Wiskott-Aldrich syndrome and TAR Syndrome
(( thrombocytopenia with absent radii
A ..... thrombocytopenia T TAR
radius R .... absent
leading to thrombocytopenia with qualitative defect

leading to Chediak Higashi syndrome


qualitative defect only

MCQ

platelets Inheritance defect


haemophilia coagulation
Von.... Haemophilia
Willebrand's disease
aggregation defect Glanzmann's disease
adhesion defect Bernard soulier syndrome
VWD
Wiskott-Aldrich syndrome and TAR
syndrome
Chediak-Higashi syndrome

Acquired disorder of platelets


non steroidal anti prostaglandins
adhesion defect platelets defect
152


..............................................................
uraemia platelets defect
Disorder of blood vessels and vascular tissue

Henoch-Schonlein purpura
Problem solving
vasculitis children and young adult

hyper sensitivity
following streptococcal infection
.. following viral infection
clinical picture




... ...
... ..

.... ....
buttocks
buttocks thigh
Henoch Schonlein purpura
.... medicine
mesenteric ischemia
... vasculitis
... glomerulonephritis .....haematuria
Haematuria painless
Henoch Schonlein purpura Ig A
nephropathy
Ig A nephropathy Henoch
Schonlein purpura
Investigations
... Non specific Investigation
153


..............................................................


ESR .... Normal complement
Most benign vasculitis
... Henoch Schonlein purpura

short term steroid


corticosteroid alone are effective for
gastrointestinal and joint involvement but
glomerulonephritis may requires treatment with both
steroid and immunosuppression
other disorder of blood vessels and vascular
tissues
collagen C scurvy
perifollicular petechiae and synthesis
C gingival bleeding
bleeding

hereditary hemorrhagic telangiectasia (osler-weber-
( Rendu disease
autosomal dominant, associated with abnormally thin
vessel walls and impaired vascular contractility with
friable blood vessels
Haemophilia

male X-linked recessive
carrier female
clinical picture
14 haemophilia ...
very mild case ....
sever cases clinical picture
154


..............................................................
circumcision
.... Haemorrhage following trauma
...
haematoma
... ..
...

Ecchymosis , haematoma after
trauma
purpura
GIT bleeding haemoarthrosis
...
Haemophilia
... ... analgesic non
steroidal anti inflammatory ....anti platelets
.... coagulation .. platelets
.. blood vessel vasoconstriction

Non steroidal
haemophilia
... .. haemophilia ...
coagulation defect
Haemophilia
MCQ
the commonest cause of coagulation defect is .
Chronic liver disease

the commonest inherited coagulation defect
..haemophilia A
A A factor 8
haemophilia B
155


..............................................................
the commonest platelets defect non steroidal

commonest platelets defect inherited Von


Willebrand's disease
....
haematoma retro peritoneal femoral
nerve
....


... ..

....
...
... subside
...
... !!!!!!! calcification
haematoma calcified
....
) (





....
... ....
dominant hemisphere

Investigation


156


..............................................................
extrinsic pathway prothrombin time
Intrinsic PTT
Von Willebrand
defect factor 8
factor 8 pathway Intrinsic pathway
.... Von Willebrand disease platelets
defect adhesion defect..... .... bleeding
time prolonged ..... PTT prolonged )...
( Von Willebrand's disease
haemophilia A
bleeding time Normal ... Platelets normal
.... PTT ... Prolonged prothrombin
time Normal
factor 8 extrinsic pathway
complication
arthropathy Hepatitis C&B HIV

severity of haemophilia
sever Moderate Mild subclinical


anti haemophilic factor Level PTT
clinically
subclinical haemophilia
Major surgery major trauma
....

avoid anti platelets


fresh frozen plasma factor 8
factor8 concentrate
.... anti fibrinolytic fibrinolytic
system
157


..............................................................
... ..... fibrin anti
fibrinolytic anti fibrinolytic
Tranxamic acid ) ( cyklokapron

EACA epsilon amino caproic acid


DDAVP Desmopressin level of factor 8



haemophilia .. Liver
liver ... factor 8 isolated defect....
Liver ............ normal
.... Liver Liver ...
immune suppression
..........
Haemophilia haematoma

C Liver cirrhosis
portal hypertension haemophilia
..... haemophilia ...
... Liver cirrhosis Portal hypertension
........... liver transplantation
liver ... cirrhotic liver cirrhosis
Liver cell failure LCF ...
Haemophilia
Isolated defect
Haemophilia

factor 9 deficiency
Haemophilia B haemophilia A
Hegman factor
Hegman factor deficiency Prolonged PTT but
with no bleeding tendency
158


..............................................................
... factor 12 deficiency
... intrinsic pathway ......
.. factors
Hegman factor
PTT Prolonged
bleeding tendency
MCQ
PTT prolonged
... bleeding tendency Hegman factor deficiency
Hegman factor ...
... Hegman factor Post
Hegman factor
... Hegman factor

Hegman factor factor 12

clinical approach bleeding tendency
coagulation defect
haematoma, haemoarthrosis and
large ecchymosis
Poor effect of compression ....
... .. ...
....
.... .... Haematoma
haemarthrosis post traumatic
History coagulation defect
coagulation defect PT or PTT or both ,
normal bleeding time

platelets defect
... mucosal bleeding petichae
haemorrhage spontaneous traumatic
159


..............................................................
coagulation defect
... haematoma..

Vascular defect
palpable purpura Post traumatic
clinical approach to acase of
bleeding tendency
coagulation defect platelet defect
vascular
... .. example

coagulation defect haemophilia


platelet defect ITP VWD
Vascular defect Henoch Schonlein
purpura

Coagulation Platelets vascular

Aplastic anaemia
problem solving
blood diseases presented sore throat

agranulocytosis
aplastic anaemia
leukaemias

agranulocytosis sore throat
throat ulcers ..... Pus .....scanty late Pus
.... neutrophils neurtophils .... Neutrophils
160


..............................................................
Pus ... Manifestation of inflammation

minimal signs of inflammation throat ulcers
..... neutrophils scanty pus
aplastic anaemia and agranulocytosis
Leukaemia
Pancytopenia aplastic anaemia
platelets RBCs agranulocytosis
secondary primary aplastic anaemia
Primary
fanconi anaemia congenital idiopathic
RBCs ... Manifestations
pallor , palpitation
recurrent infection
bleeding tendency
Monilial throat ulcers necrotic mouth
minimal signs of inflammation infection
scanty pus
Investigations
WBCs platelets RBCs
neutrophils WBCs
Lymphocytes
leucopenia ... neutrophils
with relative lymphocytosis
... neutrophils Leucopenia
WBCs neutrophils
relative lymphocytosis
.... .. Lymphocyte
total leucocytic count % 25 Lymphocyte
... neutropenia total leucocytic count
total % 25 Lymphocytes
total leucocytic count % 50 leucocytic count

total leucocytic count


161


..............................................................
normal 2000.......... 8000
...... Lymphocytes Lymphocyte percent 25
) % (
total leucocytic count .... 4000
neutrophils .... total count ..... 4000
lymphocytes 2000 percent %50
total leucocytic count
relative lymphocytosis
Lymphocytes total count ..
lymphocyte relatively percent ...
bone marrow pancytopenia bone
marrow
aplastic
. anaemia no reticulocytes ....
..... aplastic anaemia reticulocytes
bone marrow RBCs
MCQ Reticulocytosis common
... aplastic anaemia
Investigations blood bone marrow

differential diagnosis of pancytopenia



differential
Hypersplenism
folic and B12 deficiency blood
elements
aplastic anaemia

blood transfusion Immune suppression


secondary aplastic anaemia

162


..............................................................

.... pancytopenia cytotoxic drugs
choramphenicol suphonamide
non steroidal phenylbutazone penicillamine

antithyroid
chemical benzene organophosphates
viral hepatitis B&C , parvovirus , EBV HIV
paroxysmal nocturnal haemoglobinuria
stem cells RBCs complement
... platelets .. aggregation ............
WBCs .... Pancytopenia
haemolytic anaemia aplastic
anaemia paroxysmal nocturnal
haemoglobinuria
bone marrow aplasia viral infection
pure red cell aplasia
..... diamond blackfan syndrome Pure red cell
aplasia thymoma
thymoma



thymoma 3 : myasthenia gravis
pure red cell aplasia mediastinal syndrome

bone marrow failure
. bone marrow failure aplastic anaemia
pure red cell aplasia agranulocytosis Paroxysmal
nocturnal haemoglobinuria
163


..............................................................
bone marrow failure heart failure
........ Multiorgan failure
Organs ....
anaemia of chronic disease
clinical pathology
chronic disease 3 chronic
chronic inflammatory disease
........... .....rheumatoid chronic infection .T.B
......... bronchiectasis malignancy ..
malignancy
anaemia of chronic disease
haemolysis

anaemia of chronic disease chronic disase

rheumatoid non steroidal


haematemesis ..........
... haematemesis

interleukins bone
marrow bone marrow utlilization Iron
RBCs
usually )usually normocytic
( normochromic anaemia
some times microcytic
common anaemia of chronic disease
normocytic normochromic

Iron stores Normal failure of
iron utilization serum ferritin
.... microcytic microcytic hypochromic
iron deficiency anaemia iron stores

cause
164


..............................................................
trend anaemias
..... erythropoietin erythropoietin

Acute post haemorrhagic anaemia


Normocytic normochromic

2
... haemoglobin normocytic
normochromic anaemia

packed cell volume haematocrit


value
... haematocrit value immediate
...
shift fluid extra vascular
intra vascular
haemorrhage
.... haemolysis leucoytosis thrombocytosis

total leucocytic count


platelets count
... haemorrhagic anaemia normocytic
normomchromic thrombocytosis Leucocytosis
RBCs ..... bone marrow
bone marrow proliferate Leucocytosis
thrombocytosis
normocytic normochromic
acute haemorrhagic
chronic blood loss iron deficiency
anaemia hypochromic microcytic

blood
,
,
165


..............................................................

( hypercoagulable state (thrombophilia



recurrent recurrent
recurrent
recurrent venous thrombosis arterial thrombosis

In young person
) ( DVT

.......... ...
25
... ..
.... ...... ...
... ..... oral anticoagulant ....
case = thrombophilia


vascular occlusion in young person recurrent
thrombophilia
thrombophilia
example
Trousseau's syndrome ... malignancy
Para malignant.... cancer pancreas
Behcet's thrombophilia
nephrotic syndrome wasting of antithrombin 3 in
urine
S C
pregnancy oral contraceptive agents

166


..............................................................
Hypercoagulable state
..
... bed ridden .....
.... post partum DVT post partum DVT
.... clinical
.... .... ... .... ....
... 19 ...
... ....
DVT
DVT ....
.... mortality
...
...

...
..... leg elevation
... ... Post partum DVT

examples
polycythaemia rubra vera
essential thrombocytosis
myelofibrosis
.... hyperviscocity syndrome
acquired medical condition disorder
polycythaemia rubra vera myelofibrosis
thrombocytosis nephritic syndrome C
and S

deficiency of anaticoagulants
protein S and C deficieny

167


..............................................................
Factor 5 leiden

factor 5 leiden
the most common inherited hypercoagulable state

factor 5 ...... protein C
inactivate factor 5
protein C ......... anticoagulant protein C anti
coagulant ...
C ...... anticoagulant Liver
.... K dependant antgonise factor 5
anti coagulant
factor 5 Protein C
factor 5 ... factor 5 Leiden

.... factor 5 Leiden factor 5
C
factor 5 Leiden
Factor 5 Leiden usually
recurrent thrombophelbitis venous occlusion
... arterial
anti phospholipid anti body
Lupus recurrent
abortion

young person recurrent
DVT thrombophilia
Investigation
investigation
thrombophilia Gene study

ultra lap
1000 assessment
...
problem solving
DVT History previous
168


..............................................................
thrombophilia
DVT Post partum
DVT post operative bed ridden
) ( thrombophilia
.... search for the cause gene study .....
thrombophilia
assessment protein C protein S
antithrombin ... factor 5 Leiden
....
factor 5 Leiden .... protein C ... proteis S
.....
anti phospholipid anti body
12

defect
presentation of hypercoagulable state

recurrent venous thrombosis unusual
Budd Chiari Budd Chiari
.... venous occlusion ... venous occlusion
....
thrombophilia
... recurrent abortion arterial thrombosis
atherosclerosis
Investigations
PTT factor 5 protein S and C


anti coagulant


congenital deficiency treated by warfarine
anti coagulant oral for life
... protein S or C anti coagulant
169


..............................................................
oral
malignancy para malignant thrombosis
.... para malignant
oral mainly

insuline
... low molecular weight


dissmenated malignancy
heparin
oral

anti phospholipid antibody syndrome



..... antibody anti body
antibody invitro invivo


in vitro test tube
prolongation PTT impression
.... bleeding tendency
bleeding tendency
Invivo
hypercoagulation
...
antiphospholipid anti body

170


..............................................................
PTT ...
............. anti phospholipid anti body
syndrome
items 3
prolonged PTT + bleeding tendency
PTT Intrinsic haemophilia
PTT prolonged
prolonged PTT + thromobosis anti
phospholipid anti body syndrome
recurrent abortion
Lupus abortion
... Investigation lupus
antiphospholipid antibody

prolonged PTT + no bleeding tendency
bleeding thrombosis
Hegman factor deficiency

antiphospholipid
it is manifested by recurrent venous or arterial thrombosis
recurrent fetal loss placental blood supply


warfarine usually drug of choice
combination warfarine aspirin
warfarine ....

........ ......... heparin warfarine


aspirin
lupus cortisone aspirin heparin

171


..............................................................
...........
rheumatology

Hypersplenism
Pancytopenia
monocytopenia
anaemia RBCs
neutrophils
causes hypersplenism Portal
hypertension

spleen Hypersplenism
leukaemia , lymphoma
thalassaemia
commonest cause of hypersplenism
thalassaemia
pancytopenia
bone marrow .... proliferating
peripheral spleen bone marrow
proliferating
Investigation
empty blood + full (proliferating ) bone marrow
bone marrow aplastic anaemia
blood empty bone marrow empty

transfusion
splenectomy
criteria of diagnosis of hypersplenism


cytopenia of one or more cell lines
compensatory reactive bone marrow hyperplasia
172


..............................................................
splenomegally
correction of the abnormality by splenectomy

hyposplenism


splenectomy post splenectomy
sickle cell anaemia Hyposplenism
liability to infection
lab
Howell jolly bodies within RBCs Acanthocytes
RBCs Acanthoctyes
spleen
liable Infection Meningitis
vaccination
spherocytosis

rupture spleen
splenectomy blood transfusion
Meningitis
...... splenectomy
haemophilus influenza vaccine

meningitis vaccine
prophylactic penicillin
spherocytosis

complications of blood transfusion


Immunological and non immunological
Immunological
RBCs haemolytic transfusion reaction
leucocytic and platelets
173


..............................................................
platelets WBCs antigens ......
fever
non haemolytic febrile reaction
post transfusion purpura
sensitize platelets
antibody
plasma protein anaphylactic reactions

non immunological

transmission of infection

enumerate diseases can be transmitted
blood transfusion Hint
diagnosis laboratory
hepatitis markers
Hepatitis B virus marker for more details
see text
Hepatitis C , HIV
CMV EBV Ig G Ig M
malaria blood film
trypanosomiasis bone marrow
toxoplasma toxoplasmid test
lymph node biopsy

iron overload
....... massive transfusion Massive
transfusion blood volume

...
..... massive transfusion electrolyte
disturbance

acute haemolysis fever , rigors
174


..............................................................
haemoglobinuria
acute haemolysis renal failure
renal failure acute tubular necrosis
management blood transfusion
re group



reaction
.......
febrile non haemolytic
platelets WBCs
.... Haemolysis
... ... ......
paracetmol injection ............ Infusion
antihistaminic .....

Delayed haemolytic transfusion reaction


haemolytic reaction 7 5 Late
Haemolysis immediate
some times ................ late

acute haemolysis fever ,
rigors , loin pain and renal failure (acute tubular
( necrosis

fluid and alkaline uraemia

) ....
haematology haemoglobin
8 mean corpuscular volume
... fever sore throat
175


..............................................................
responding antibiotic blood disease
leukaemia aplastic anaemia ...
... ...
(
!!!! !!!!


... blood
components and products
...


whole blood
470
63 anticoagulant 4
MCQ
2 4 6 4-
4-
Shelf-life
shock

fluid
kidney

kidney ... ...... 4
.............. 180/100 heart
........ ............. fluid


suppurative lung disease saline
Pus anaerobes
........

..... Pulmonary edema kidney


176


..............................................................
180/100 . 200/120 kidney
.....
) (

... ....

High pressure
.......

...
final
...

packed red cells


whole blood volume shocked
Packed red cell )
( ......
) (
volume over load
haemoglobin volume
whashed red cell concentrates
... packed RBCs
RBCs


) (
RBCs washed RBCs
... RBCs

Autologous transfusion
... ...... ...

177


..............................................................

2
15
autologous
bone marrow

2 predeposit


Preoperative haemodilution
dilution immediately
...
saline .....

.........

Blood salvage

.......

..........

platelet concentrates
....
22
essential sever thrombocytopenia
whole blood
granulocyte concentrate

whole blood fresh
178


..............................................................
fresh ....
...... ...
blood group
....
...

... ....






fresh frozen plasma


replacement of coagulation factor
cryoprecipitate

factor 8 Von Willebrand factor fresh
frozen
cryoprecipitate factor 8 and Von Willebrand factor

factor 8 and factor 9 concentrate
immunogloulins
Human albumin


Myelodysplasia
179


..............................................................

bone marrow Hyper
active

macrocytosis
hypersegmentation Neutrophils
....... myelodysplasia myelodysplasia
... bone marrow bone marrow hyper
active
RBCs
WBCs Hypersegmented
presented mainly
refractory anaemia
Leukaemia 5-4-3
chronic myelomoncytic leukaemia
refractory anaemia with excess of blasts
refractory anemia with excess of blasts in transfusion
myelodysplasia
pre leukaemia
bone marrow
leukaemia
refractory anaemia bone marrow examination
myelodysplasia myelo bone marrow
dysplasia abnormal growth proliferate

lekaemia
diagnosis macrocytosis
management blood transfusion erythropoietin
bone marrow transplantation

bone marrow transplantation


indication
thalassaemia major
sickle cell anaemia
180


..............................................................
Myelodysplasia
leukaemia
Lymphoma
aplastic anaemia

method
bone marrow bone marrow
iliac bone Needle
bone marrow )
( heparin fibrinolytic
bone marrow
intra venous
bone marrow bone marrow


bone marrow



complications
infection Graft versus host disease
((GVHD
..... Host bone
marrow
graft ......... bone marrow
bone marrow bone
.... marrow bone marrow
bone marrow WBCs WBCs
WBCs Bone marrow

Graft versus host disease graft
host host

cytotoxic activity of the donor T lymphocytes
sensitized immune host
acute chronic
acute can affect skin , liver , gut
181


..............................................................
collagen disease chronic

DIC
Disseminated intravascular coagulopathy
wide spread coagulopathy due to activation of
coagulation process
extrinsic Intrinsic
activation DIC
extrinsic pathway Intrinsic
etiolgogy and pathogenesis
activation of intrinsic coagulation pathway by ****
endothelial damage e.g. gram negative sepsis ,
meningocoocemia and viremia
activation of extrinsic pathway by abnormal entery of *****
tissue thromboplastins into the circulation e.g. in
obesteteric complications, carcinomatosis and massive
trauma
DIC
Initiated by stimuli in the systemic circulation DIC
that activate the intrinsic or the extrinsic pathways
microcirculation thrombin
organ damage organ damage
renal failure
consumption of coagulation factors ,
platelets
bleeding tendency

wide spread coagulopathy DIC
extrinsic pathways intrinsic triggering Due to
extrinsic Intrinsic coagulation pathway

182


..............................................................
organ failure vascular occlusion
consumption of platelets coagluation
bleeding tendency and factors of coagulations
stimulation of excessive blood clots .N.B
fibrinolytic system
excess fibrin excessive blood clot
fibrinolytic system stimulation
fibrin fibrin fibrinolytic system
(degradation product (FDP
bleeding tendency FDP

wide spread coagulopathy DIC

extrinsic intrinsic stimulation due to
pathway
vascular occlusion
renal failure organ failure
brain , heart , brain infraction
kidney
consumption of platelets and coagulation
factors
bleeding tendency
vascular occlusion ........organ failure
bleeding tendency
excessive ) excessive blood clot
.N.B
stimulation of fibrinoltyic system ( fibrin
FDP fibrinolytic system
3 DIC

septecaemia
haemorrhage
vascular occlusion
fat embolism septecaemia .......
183


..............................................................
malignancy... DIC
massive trauma

vital ....vascular occlusion vital
Death Is Coming
...... .... DIC
very mild
...
subclinical DIC

extensive vascular occlusion




clinical pictures
acute cases amionetic fluid embolism
sepsis leukaemia N3 depletion
.... dominant consumption
chronic intra utrine fetal
death ...

chronic DIC
chronic DIC vascular occlusion
consumption
Investigations
acute chronic
acute

prothrombin time prolonged
extrinsic pathway
PTT prolonged intrinsic pathway

bleeding time prolonged PT PTT


prolonged DIC
184


..............................................................
depletion acute
chronic
..... acute chronic Mild
platelets factors of coagulations

DIC
High acute or chronic DIC
high FDP
FDP acute chronic
of choice
DIC platelets
consumed factors coagulations consumed
extrinsic pathway Intrinsic pathway PT
PTT prolonged
FDP of choice

in acute DIC
blood products High risk of
bleeding

Platelets factors of coagulations
coagulation ...
fueling the fire ....
.... Platelets ... 5,000 ... factor of
coagulations
blood product platelets
plasma

chronic DIC
... heparin
Of choice

vascular occlusion heparin
185


..............................................................


acute consumption
replacement Platelets
factors of coagulations
chronic anticoagulant


blood 2009

**** !!!!!
"..... ,
" *****

186

You might also like