This aim is achieved by the use of intravenous infusion of a factor VIII concentrate. As a general rule most bleeds can be arrested by increasing the plasma level of the deficient factor to 30 to 60% of normal.
Cryoprecipitate is another source of Factor VIII.
The 190 kb human Factor VIII gene was cloned in 1984 and biosynthetic Factor VIII is undergoing clinical trials.
Mild haemophiliacs may be treated with iv vasopressin (DDAVP) which increases levels of circulating Factor VIII, and/or tranexamic acid - an antifibrinolytic agent.
Haemophilia B:
In haemophilia B correction of the bleeding tendency is achieved by infusion of factor IX concentrate. As with haemophilia A most bleeds can be arrested by increasing the concentration of factor IX to 30 to 60% of normal. Factor IX concentrates contain additional proteins such as activated coagulating factors which increase risk of thrombosis and thus must be used carefully. DDAVP is not effective.
General:
Correctly treated, haemophiliacs can enjoy a normal life span and social / professional ambitions.
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