what is bleeding disorder? classification hemophilia clinical features management von willibrand disease types diagnosis management

1. z
BLEEDING
DISORDERS

2. z
What is it?
•BLEEDING DISORDER is an
acquired or inherited
tendency to bleed
excessively.

4. CLASSIFICATION
VESSEL
DISORDER
PLATELETS
DISORDER
COAGULATION
CASCADE

5. VESSEL DISORDER
 Vitamin C Deficiency
 Bacterial Infection
 Viral Infection
 Acquired
 Easy Bruising
 Steroids

6. PLATELETS DISORDER
 Thrombocytopenia
 Chemotherapy
 Aplastic Anaemia
 Viral Infection
 Thrombocytopathy
 Kidney Failure

7. COAGULATION CASCADE
Hereditary:
•Hemophilia A&B
•Von Willebrand's
Acquired :
•Liver Disease
•Drug- Heparin
•Disseminated Intravascular Coagulation

8. z
COAGULATION FACTOR
DEFICIENCY
Hemophilia A is due to a
deficiency of factor 8
Hemophilia B is due to
deficiency of clotting factor 9
Inherited x-linked disorder

9. z
The clinical severity depends on the level of
factor 8 = severity of condition
•Mild = > 5%
•Moderate = 1- 5%
•Severe = < 1%

10. z
LABORATORY INVESTIGATIONS
COAGULATION TESTING
Prolonged activated partial thromboplastin time
Normal prothrombin time
Normal bleeding time
Reduced level of factor 8

11. z
General Management
Factor replacement Administration of
factor 8
concentrated by
intravenous
infusion.
•Minor bleeding:
factor 8:C level
should be raised to
20 – 30 %
•Severe bleeding:
factor 8:C level
should raised to at
least 50%

12. z
VON WILLBRANDS DISEASE
Hereditary coagulation abnormality caused
by either;
Reduced level of vwf
Abnormality in vwf
Due to point mutation or major deletion

14. CLINICAL FEATURES
 typically there is mucous membrane bleeding
 the severity of symptoms variable with types
type 1,2 usually mild symptoms
type 3 severe symptoms
LABORATORY INVESTIGATIONS
 the bleeding time is prolonged
 apt is prolonged
 factor 8 and vwf is low
 platelet count is normal

17. z
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