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c. Treatment options :
1. Fresh frozen plasma (FFP)
2. Cryoprecipitate
3. Factor concentrates
5. Complication : Arthropathy, Inhibitors,
Liver disease, HIV infection.
6. Interdisciplinary care hemophilia center
medical care, psychosocial care and
genetic counseling
Finding
Hemophilia A
vWF disease
Normal
Prolonged
Decreased
Decreased
Normal
Decreased
Normal
Decreased
4. Therapy
a. dDVAP
b. Cryoprecipitate
c. F VIII concentrates
3. Diagnosis
- severe : prolonged PT and PTT
- early or milder def. : prolonged PT
4. Therapy
- Parenteral vit. K
- FFP
B. Liver disease
1. Etiology
a. decreased synthesis of coag. factors
b. Vit. K def
4. Therapy
- trial of parenteral vit K therapy
- Replacement therapy FFP
C. Clotting factor inhibitors autoantibodies
1. Inhibitors in hemophilia
2. Inhibitors in patients without preexisting
bleeding disorders
a. Etiology : drugs, autoimmune or
lymphoproliferative disorders,
spontaneous a.coagulants
COAGULATION REGULATION
AND
HYPERCOAGULABLE STATES
B. Fibrinolitic system
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
II. THROMBOTIC DISORDER
A. Congenital thrombotic disorder
1. AT III deficiency
2. Protein C deficiency
3. Protein S deficiency
b. Pulmonary embolism
c. Arterial thrombosis
d. Other
4. Therapy
a. Agents treatment and prevention
1. Anticoagulants :
- Heparin
- Warfarin
2. Thrombolytic agents :
- Streptokinase
- Urokinase
- t-PA
b. Treatment approaches
1. Deep vein thrombosis : >> heparin,
thrombolytic agent
2. Pulmonary embolus : heparin, thrombolytic
agent
3. Myocardial infarction : thrombolytic agent
4. Peripheral artery and catheter thrombosis :
thrombolytic agent.
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
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