AND
HEMORRHAGIC DISORDERS
Prekallikrein, HMWK
fibrinogen.
INTRINSIC SYSTEM
HMWK
XII XII a
Kallikrein
VII
IX IXa + VIII TF
Ca 2+ Ca 2+ Ca 2+
PL
X Xa + V
Ca 2+
PL
Prothrombin Thrombin
Fibrinogen Fibrin
A. Screening Test
1. Partial thromboplastin time (PTT) and
activated partial thromboplastin time (aPTT)
2. Prothrombin time (PT)
3. Quantitative fibrinogen
4. Thrombin time (TT)
5. Screening test for factor XIII
a. Pharmacologic therapy :
b. Replacement therapy :
1. Physiology of vWF :
- HMW glycoprotein, 250 kD
- In plasma and in platelets
- as a carrier protein for coag. F VIII
- important for primary hemostasis
- mediates adhesion of platelets to the s.endot
- vWF – F VIII complex : F VIII : C, F VIII : Ag,
F VIII : Cag, F VIII : RCof.
2. Clinical features : >> mucous membrane
bleeding, epistaxis, menorrhagia
3. Diagnosis :
- Comb. of prolonged BT and a decreased
F VIII : C level classically
- Comb. of abnormalities of the functional
measures of the vWF – F VIII complex
variants of vWF (table 2)
- Ristocetin aggregation
- Diff. of type F VIII multimer analysis :
type I, IIA, IIB, IIC, III.
Table 2. Laboratory Findings in Hemophilia A
and Severe vWF Disease
1. Activation :
a. Intrinsic activation : a poorly understood
mechanism of activation
b. Extrinsic Activation : Tissue plasminogen
activator (t-PA), urokinase
c. Exogeneous (therapeutic) activation :
streptokinase, urokinase and t-PA
2. Sites of action
a. Fibrinogen
b. Fibrin
Secondary fibrinolysis
Bleeding tendency
Ischemic Microangiopathic
tissue hemolytic
damage anemia
b. thrombotic lessions
products (FDP)
4. Therapy :
a. Low grade DIC treatment may not be
necessary
b. Clinically significant bleeding
replacement of depleted coagulation
factors and cells
c. Thrombosis : heparin
B. Thrombotic thrombocytopenic purpura (TTP)
C. Heparin thrombocytopenia