ABSTRACT
Treatment of haemophilia A is with factor VIII concentrate either as a regular infusion or when actively bleeding. Patients receiving regular infusions have higher factor VIII levels and a better quality of life, but are at higher risk of developing antibodies to the extrinsic factor VIII, which reduces its efficiency. Patients who receive factor VIII only when bleeding are less likely to form antibodies, but are at increased risk of bleeding. Patients with mild disease may be treated with desmopressin (DDVAP), which releases factor VIII from internal stores. Factor VIII concentrate should be given prior to invasive procedures such as tooth extraction and surgery. Haemophilia B is treated with factor IX concentrate. It should be noted that many patients with haemophilia who
Stephen Christmas, the first person described to have factor IX deficiency (1947-93). He eventually died from AIDS, transmitted via a transfusion. The original case report of Christmas disease was reported in the Christmas edition of the British Medical Journal in 1952.
