HEMATOONKOLOGI

BIMBEL UKDI MANTAP

dr. Anindya K Zahra
 1
RBC
Anemia?
Approach to Anemia: MCV! ADB

Penyakit
kronik
Mikrositik
Besi Serum
Hipokromik
Thalassemia
MCV 
N
Sideroblastik

Anemia
hemolitik
Anemia Normositik
normokromik
Restikulosit 
Perdarahan
MCV Normal Akut

Aplastik
Defisiensi
folat N/
Makrositik Leukemia, etc
Defisiensi
MCV  B12
MDT
Anemia Mi-Hi
Anemia Mi-Hi
 ADB

Angular cheilitis

Smooth tongue

Koilonychia –brittle spoon-shaped nail

 ADB

ADB: Mikrositik hipokromik Normal

(central pallor >>), Pencil cell (+)
 Terapi ADB
First line: Oral iron therapy

• Ferrous sulphate 3x200 mg

• Ferrous sulfate 325 mg = 65 mg elemental iron
• Ferrous gluconate 325 mg = 38 mg elemental iron.

Bone marrow response to iron is limited to 20 mg per day of

elemental iron

Target: Hb increase of 1 g/dL every 2-3 weeks

Hb corrected  iron stores return to normal (~4 months)

 Oral Iron Therapy

 
• Antasida • Daging
• Fitat (pada sereal) • Senyawa sitrat
• Tanin (pada teh) • Fruktosa
• Fosfat • Asam askorbat

• Efek samping Fe  Gastric upset. Intoleransi terutama berkaitan

dengan besarnya kadar zat besi terlarut yang ada dalam lumen usus
 dapat dicegah dengan memberikan dosis awal yang rendah.
Anemia Mi-Hi
Thalassemia akan dibahas di
anemia hemolitik
 Anemia Sideroblastik
• Genetic (X-linked
or AD) or acquired
(myelodysplasia
syndrome)
• Sideroblast:
nucleated
erythroblast
• “Ring”: iron in
perinuclear
mithocondria
• Iron (+) but
cannot corporate
it to Hb

Bone marrow aspirate: ring sideroblast

Anemia Mi-Hi
 8
Megaloblastic Anemia
Anemia megaloblastic
 9
Anemia Hemolitik
Curiga anemia hemolitik:
• Klinis: Anemia, Jaundice, Splenomegali
• Lab: Retikulosit , Bilirubin indirek 
 Hemolisis

Letak Penyebab

Extravascular Intravascular
Intrinsik Extrinsik
(90%) (10%)

RE system Membran Autoimun

Enzim Infeksi

Hemoglobin Microangiopathy
Anemia Hemolitik: Defek Intrinsik

Hereditary Osmotic fragility

Membran
spherocyte test

Intrinsik Enzim G6PD deficiency G6PD assay

Hb
Thalassemia
elektroforesis
Hemoglobin

Sickle cell
Anemia Hemolitik: Defek Intrinsik

Hereditary Osmotic fragility

Membran
spherocyte test

Intrinsik Enzim G6PD deficiency G6PD assay

Hb
Thalassemia
elektroforesis
Hemoglobin

Sickle cell
Membranopathy

Hereditary Spherocytosis
• MDT  Spherocytes
• Osmotic fragility test
• Slenectomy often very effective
Anemia Hemolitik: Defek Intrinsik

Hereditary Osmotic fragility

Membran
spherocyte test

Intrinsik Enzim G6PD deficiency G6PD assay

Hb
Thalassemia
elektroforesis
Hemoglobin

Sickle cell
Enzymopathy
G6PD Deficiency
G6PD Deficiency
G6PD Deficiency
Anemia Hemolitik: Defek Intrinsik

Hereditary Osmotic fragility

Membran
spherocyte test

Intrinsik Enzim G6PD deficiency G6PD assay

Hb
Thalassemia
elektroforesis
Hemoglobin

Sickle cell
 Hemoglobinopathy
Hemoglobin Deffect

Thalassemia Sickle cell disease

Hb elektroforesis

Thalassemia: microcytic hypochromic anemia,

anisositosis, poikilositosis, target cell
 What is thalassemia?
• Inherited disorders
• Defective hemoglobin chains
• The two main types:
– Alpha
– Beta  more severe
• Hb Elektroforesis  HbA2  & HbF 
Suspect thalassemia if:
• Family history (+)
• Microcytic anemia
• Jaundice
• Bone deformities
• Splenomegaly
• Appearance early in life
a Thalassemia minor: often no target cells, but an increase in the number of small erythrocytes
(shown here in comparison with a lymphocyte), so that sometimes there is no anemia.
b More advanced thalassemia minor: strong anisocytosis and poikilocytosis (1), basophilic
stippling (2), and sporadic target cells (3).
Thalassemia
 Splenomegaly

Splenomegaly &Extramedullary hematopoiesis

FACIES RODENT
 Thalassemia
Chronic
Iron overload Tissue damage
hemolysis

Mechanism

• Excess iron  free hydroxyl radicals  ROS

• Insoluble iron complexes  deposited in
body tissues

Clinical sequelae of iron overload

• Pituitary → impaired growth

• Heart → cardiomyopathy, heart failure
• Liver → hepatic cirrhosis
• Pancreas → diabetes mellitus
• Gonads → hypogonadism, infertility
 Iron Chelating
(Deferoxamine/Deferiprone/ICL670 )

+ IRON CHELATING

TRANSFUSI PRC BERKALA

*
*
Anemia Hemolitik: Defek Ekstrinsik

Warm
Autoimun
Cold

Prosthetic
Extrinsik Microangiopathy
valves etc

Infeksi Malaria, etc

 Autoimmune Hemolytic Anemia
Warm & Cold AIHA
Warm Cold
Maximally bind 37°C 0° to 4°C
RBCs at
Clinical Acute and severe Post infectious, idiopathic
Collagen disease, idiopathic
Younger age group Older age group
Mediated by IgG IgM which fixes complement
autoandibodies (C3)
Mechanism IgG-coated RBCs  partially IgM + RBC  activate
ingested by the macrophages of complement  C3 coated RBC
the spleen  microspherocytes  agglutination 
 extravascular hemolysis intravascular hemolysis
Treatment Corticosteroid Avoidance of cold
Splenectomy
Transfusion therapy in AIHA is challenging, and the most compatible
red blood cells should be given
Coombs’ Test
 AIHA
Warm AIHA:
spherocytes
 Microangiopathic Anemia

Schistocytes and microspherocytes noted on the blood smear

Cause:
• Microvascular disease (DIC, TTP etc)
• Heart valve prostheses
• Trauma / implanted devices
 16
Deep Vein Thrombosis
• VIRCHOW
• FR
• WELL’S
Well’s Score
 18
Komponen darah

Platelet Fresh Frozen

Cryoprecipitate
Concentrate Plasma
Fibrinogen, von
All coagulation Willebrand factor,
Trombosit
factor factor VIII, factor XIII
and fibronectin.

Haemophilia A, Von
Multiple coagulation
Trombositopenia, Willebrand’s
factor deficiency,
profilaksis (operasi), disease,
DIC
Hipofibrinogenemia
 20
ITP
 Terapi ITP
ITP: antiplatelet antibody  platelet destruction

Kortikosteroid
• Indikasi: AT<20.000-30.000 atau AT <50.000 dengan
perdarahan/risiko perdarahan (HT, peptic ulcer)
• Prednisone 1-1,5mg/kg/hari

IvIg
• Indikasi: severe, life-threatening bleeding, atau anak dengan AT
<20.000 dengan perdarahan minor

Platelet
• “Not indicated unless there is significant bleeding. In ITP transfusion
increments are usually poor and platelet survival is short”
 PT

APTT

‘coagulation
cascade’

TT ‘waterfall’

*
Fibrinolysis system
Patophysiology of DIC
Clinical manifestation of DIC
 Lab diagnosis of DIC
DIC
• Screening : bleeding time (>>) , PT (>>), APTT
(>>) , platelet count (<<), fibrinogen (<<)
• Diagnosis: Fibrin degradation product (FDP)
(>>), D-dimer (>>), AT III (<<)
Polisitemia
Polisitemia
 Kriteria PV oleh PVSG
KATEGORI A KATEGORI B

1. Total volume RBC ≥ 36 ml/kg pada 1. Trombositosis >400.000/mm3

pria, ≥ 32 ml/kg pada pria 2. Leukositosis >12.000/mm3 (tanpa
2. SaO2 > 92% demam/infeksi)
3. Splenomegali 3. Leukocyte alkaline phosphatase score
>100
4. Serum vitamin B12 >900 pg/ml atau
serum UB12BC >2.200 pg/ml

KRITERIA DIAGNOSIS PV

A1 + A2+ A3 atau A1 + A2 + 2 kategori B

Polsitemia Vera
KEGANASAN HEMATOLOGI
 Leukemia
CBC Acute Chronic
Hb  (anemia  (anemia)
AL  (leukositosis)  (leukositosis)
AT  (trombositopenia) - N/
-  in CML blast crisis
Diff count blast cells (nucleoli (+)) immature granulocytes (all
stage of maturation)
Myeloid (AML) Lymphoid (ALL) Myeloid Lymphoid CLL
(CML)
80-90% case Adults >55 yo
Adult & children Children>> Philadelphia Limfositosis
Myeloblast >20% Limfoblast >20% chromosom >50rb
Auer rod (+)

*Pansitopenia may present in the early sign of leukemia

 AML M1:

AML without
maturation

Myeloblast > 80-90%

Auer rod

nucleoli
 AML-M3
promyelocytes

Multiple Auer rod

• Hypergranular: consist of procoagulant

(promote coagulation activity) induce DIC
 • ALL-L1: small uniform cells

 ALL-L3: large varied cells with

strongly basophilic cytoplasm &
 ALL-L1: uniform cell, small blast cell
vacuoles (bubble-like features)
with scanty cytoplasm

 ALL-L2: varied cell, large blast cells

with prominent nucleoli & cytoplasm
and with more heterogeneity
 Chronic Blast
several years Accelerated
phase transformation
phase
triphasic

biphasic several years

• Fase:
– Kronik: blast <5%
– Accelerated: blast >15%
– Acute/Blast crisis: blast >30% (mirip AML)
Lymphoma: Hodgkin & Non-Hodgkin(85%)

B symptoms (+) in Hodgkin.

NHL  B symptoms (+) in
advance & late stage
Hodgkin Lymphoma
“Owl’s Eyes”
Reed Stenberg cell (+)
Hodgkin Lymphoma
Reed Stenberg Cell –Owl’s Eyes
 Biopsy
Excisional or incisional biopsy
• In this type of biopsy, a surgeon cuts through the skin to remove the entire tumor
(called an excisional biopsy) or a small part of a large tumor (called an incisional
biopsy).

Enucleation
• surgical removal of a mass without cutting into or dissecting it. Eg: eye, oral pathology,
uterine fibroids (without hysterectomy)

FNA
• does not require an incision

Core biopsy
• uses needles that are slightly larger than those used in FNA
• Local anasthesia
• Sometimes uses a special vacuum tools to get larger core biopsies from breast tissue
Terima Kasih