Correct the bleeding tendency

Haemophilia A:

• 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.