ABSTRACT
Haemophilia A (factor VIII deficiency) is a sex-linked recessive condition which occurs in 1 in 20 000 males. A family history is obtained in over 50% of cases. Christmas disease (factor IX deficiency=haemophilia B) is also sex-linked recessive but is much less common. These coagulopathies may present in infancy with excessive superficial bruising, or bleeding following circumcision. Once the child is mobile, haemarthroses are common and bleeding post tonsillectomy may be life-threatening. Bleeding from cuts or from mucosal surfaces (e.g. epistaxis) is less common, but haematuria does occur. Female carriers are asymptomatic but can be detected by reduced factor VIII or IX activity.
