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