ABSTRACT
HAEMOPHILIA Haemophilia A is a congenital disorder of coagulation, characterized by deficiency of factor VIII in the blood. Deficiency of factor IX results in an identical clinical condition known as haemophilia B (also known as Christmas disease). Haemophilia is encountered in all racial groups, with an incidence of approximately one in 10,000 males. The clinical picture is dependent on the degree of deficiency of the coagulation factor in the blood: severe haemophilia is associated with a level of less than 1% of normal. The hallmark of severe haemophilia is recurrent and spontaneous bleeding into joints, principally the knees, elbows and ankles. Repeated bleeding into joints can, in the absence of treatment, result in disabling arthritis at an early age. Bleeding into muscles and soft tissues is also frequently seen.
