PIS SPONTAN TERKAIT

GANGGUAN HEMATOLOGI

2 kategori besar
Didapat (Acquired)
Dari lahir (congenital)

Didapat (Acquired)
Drug (antiplatelet, anticoagulant,
thrombolytic)
Thrombocytopenia

antiplatelet

Coagulation cascade, the traditional concept

Fibrinolysis

Components of the fibrinolytic system:

- plasminogen and plasmin

The fibrinolytic system

Plasminogen activators:
- tissue plaminogen activator (tPA)
- urinary plaminogen activator (uPA)
- exogenous plasminogen activators
(streptokinase)

trombositopenia

Hemofilia

Congenital

Coagulation Disorders

HemophiliaB
(Christmas
Disease)results
from
deficiencyoffactor
IX
Heparinisacofactor
thatallows
antithrombinIIIto
inactivatethrombin
andFactorXa

HemophiliaA
(classic)isdueto
reducedamountor
reducedactivityof
FactorVIII
Thrombomodulin
bindstothrombin,
makingitan
anticoagulantwhich
thenactivatesanti
coagulantproteinC.
ProteinCcleave
factorsVaandVIIIa

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.

Terima
Kasih..

10/21/15