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Hemostasis & Bleeding disorders

Dairion Gatot, Savita Handayani

Divisi Hematology -Onkology Medic


Internal Departement of Medical Faculty of North
Sumatera University /
Haji Adam Malik General Hospital,
Medan 2011
HEMOSTASIS, (Virchows Triad)
Function of HEMOSTASIS

ARREST BLEEDING
MAINTAIN BLOOD IN FLUID STATE
HOMEOSTATIC HEMOSTASIS
Fisiologic funtion Maintain blood in fluid state
Hemostatic Balance
Hemostasis an equilibrium of physiological activators and
inhibitors of coagulation
Theequilibrium
The equilibriumisisfragile
fragileand
andseveral
several
causes put
causes putpatient
patientatatrisk
risk

PAI-1 Prot. S

Antiplasmin Prot. C

Tissue factor* TFPI


Fibrinolytic
Clotting Factors System
ATIII

Procoagulant Anticoagulant
HEMOSTASIS
Primary Hemostasis
Blood vessel contraction
Platelet Plug Formation
Secondary Hemostasis
Activation of Clotting Cascade
Deposition & Stabilization of Fibrin
Tertiary Hemostasis
Dissolution of Fibrin Clot
Dependent on Plasminogen Activation
Hemostasis Lab Tests
CBC-Plt
BV Injury BT,(CT)
PT
Tissue PTT
Neural Platelet Factor
Adhesion
and
Activation

Blood Vessel Platelet Coagulation


Constriction Aggregation Cascade
Primary hemostatic plug

Reduced
Fibrin
Blood flow formation

Plt Study
Morphology
Stable Hemostatic Plug
Function
Antibody
The primary haemostatic system:
haemostasis and platelet plug formation

Primary haemostasis
Platelet aggregation

trombosit
Adhesion
endothelial cells
Activation
sub endothelial tissue

Vascular Aggregation
injury
White clot

Formation of
platelet plug

exposed sub
endothelial tissue
The haemostatic system:
secondary haemostasis and clot
formation
Prothrombin Thrombin Activation of the
Factor coagulation cascade
Intrinsic
Xa
Extrinsic Fibrinogen Fibrin
leads to generation of
pathway pathway thrombin and, in turn,
fibrin

Coagulation cascade
leads to clot formation

Clot
growth
Fibrin threads
The haemostatic system:
Tertiary haemostasis and clot formation
Clinical Manifestation of Hemostatic
defect
Ptechia
Purpura
Echymosis
Haematoma
Haematemesis
Melena
Haemathrosis
Haematophysis
Hematuria
Epistaxis
Gum bleedeing
Petechia : a minute, rounded spot of haemorrhage on a
surface, such as skin, mucous membrane, serious
membrane
Purpura : a condition in which haemorrhage occur in the
skin
Ecchymosis: extravasasion of blood into the
subcutaneous tissue. It is marked by purple discoloration
of the skin, the color gradually changing to brown green
and yellow
Hematemesis : the vomiting blood
Hemoptysis : the spitting of blood from larynx ,pharynx,
trachea, bronchi or lung
Hematoma : a focalized extravasation of blood which
soon clot to form a solid mass and readily became
encapsulated by connective tissue
Melena : the discharge of stool colored black by altered
blood
Hemathrosis : extravasation of blood into a joint
Hematuria : the discharge of urine containing blood
Epistaxis : bleeding from nose
Hematoschezia : the discharge of stool colored red or
brown
Disorders of Hemostasis
Vascular disorders
Scurvy, easy bruising, Henoch-Schonlein purpura.
Platelet disorders
Quantitative - Thrombocytopenia
Qualitative - Platelet function disorders Glanzmans
Coagulation disorders
Congenital - Haemophilia (A, B), Von-Willebrands
Acquired - Vitamin-K deficiency, Liver disease
Mixed/Consumption: DIC
VASCULAR
Vascular
Purpura, echymosis
Connective tissue Ehler-Danlos Syndrome
Aging process senile purpura (Batemans disease)
Infectious
Meningococcus Rocky Mountains syndrome
typhoid fever Roseola spot
Vit C deficiency scurvy
Immunologic Henoch-Schonlein purpura
fragility capillary infectious, vasculitis
Senile Purpura
Henoch-Schonlein purpura

20y Male, fever, painful symmetric polyarthritis for a day. During the next two days,
edema and palpable purpura developed.
Henoch-Schonlein purpura
Immune disorder
Children
Follows infection
Petechiae with
edema and
itching.
9 Maret 2004
9 Maret 2004
9 Maret 2004
9 Maret 2004
Vascular
Non palpable purpura
senile purpura
scurvy
use corticosteroid
Palpable purpura Henoch-Schonlein
syndrome
THE ROLE OF PLATELET IN
HEMOSTASIS
THE ROLE OF PLATELET IN HEMOSTASIS
Platelet dysfunction:
Inherited Disorders:
Bernard-Soulier disease
large platelets, failure of adhesion
Glanzmanns thrombasthenia
normal size, failure of aggregation
Acquired Disorders:
Drugs : Aspirin
Alcohol
Uremia,
Platelet dysfunction

Quantitatif : - thrombocytemia
- ITP
- aplastic anemia
- DHF
- acute leukemia
- hypersplenism
Platelet Disorders - Features:

Mucocutaneous bleeding
Petechiae, Purpura, Ecchymosis.
Spontaneous bleeding after trauma
CNS bleeding (severe, plt)
Prolonged bleeding time (BT)
Dengue Hemorrhagic fever

Platelet deficiency..
Petechiae
(typical of platelet disorders)

Do not blanch with pressure


(cf. angiomas)
Not palpable
(cf. vasculitis)
Idiopathic Thrombocytopenic
Purpura (ITP)
Acute - children (post infection)
Chronic - adults ( females, 20-40 yrs)
autoimmune disorder
antiplatelet antibodies (IgG)
IgG coated platelets removed by spleen
Usually megakaryocytes in BM
COAGULATION DISORDERS
Coagulation Disorders
HemophiliaA
(classic)isdueto
reducedamountor
reducedactivityof
HemophiliaB FactorVIII
(Christmas
Disease)results
from Thrombomodulin
deficiencyoffactor bindstothrombin,
IX
makingitan
Heparinisacofactor anticoagulantwhich
thatallows thenactivatesanti
antithrombinIIIto coagulantproteinC.
inactivatethrombin ProteinCcleave
andFactorXa factorsVaandVIIIa
Coagulation disorders:
Deficiencies of Clotting factors
Onset - delayed after trauma
Deep bleeding
Into joints - Hemarthroses
Into deep tissues Hematoma
large skin bleed Ecchymoses
Ecchymoses

(typical of
coagulation factor
disorders)
Coagulation Disorders
Laboratory findings:
Normal bleeding time & Platelet count
Prolonged prothrombin time (PT)
deficiencies of II, V, VII, X
Prolonged time (aPTT)
all factors except VII, XIII
Mixing studies - normal plasma corrects PT or
aPTT
Factor VIII Deficiency
Classic hemophilia (hemophilia A):

X-linked disorder (affects 1 males)


Most common - severe bleeding
Spontaneous hematomas < 1, 5, 75%
Abnormal aPTT Intrinsic path.
Diagnosis - factor VIII assay
Treatment - factor VIII concentrate
Cryoprecipitate (less desirable)
Concentrat factor VIII Koate
Recombinat factor VIII
Factor IX Deficiency
Christmas disease (Hemophilia B):

X-linked recessive disorder


Indistinguishable from classic hemophilia (F VIII)
Requires evaluation of factor VIII and IX activity
levels to diagnose
Treatment - factor IX concentrate
Cryoprecipitate if factor IX unavailable
Von-Willebrand Disease:
Coagulation + PLT disorder:

Congenital disorder
Deficiency of vWF molecule
Part of FVIII,
Mediates platelet adhesion
Prolonged Bleeding time
Low Factor VIII & long aPTT
Mucocutaneous bleeding
Von-Willebrand Disease:

vWF: F-VIII & Plt function.


Defective Platelet Adhesion
Skin Bleeding
Prolonged Bleeding time.
Low Factor VIII levels.
Secondary Hemostatic Disorders

Acquired coagulation disorder:

Vitamin K deficiency
- neonates - decreased intestinal
flora and dietary intake
- oral anticoagulants (coumadin)
- fat malabsorption syndromes

Required for factors II, VII, IX, X


Prolonged PT and aPTT
Combined Primary and Secondary
Hemostatic Disorders

Severe Liver Disease:


Primary - dysfunctional platelets and/or

thrombocytopenia ( BT)
Secondary - decrease in all coagulation factors

except vWF ( PT, aPTT)


Vitamin K will promote synthesis of factors II, VII,

IX, X
Clinical Features of Bleeding Disorders

Platelet Coagulation
disorders factor disorders

Skin Deep in soft tissues


Site of bleeding Mucous membranes (joints, muscles)
(epistaxis, gum,
vaginal, GI tract)
Petechiae Yes No
Ecchymoses (bruises) Small, superficial Large, deep
Hemarthrosis / muscle bleeding Extremely rare Common
Bleeding after cuts & scratches Yes No
Bleeding after surgery or trauma Immediate, Delayed (1-2 days),
usually mild often severe
Platelet Coagulation

Petechiae, Purpura Hematoma, Joint bl.


Summary