Pathogenesis and Management of Acquired Haemophilia

The development of auto-antibodies to factor VIII results in a severe bleeding disorder, which has a mortality of up to 22%. It is predominantly a disease of the elderly, and probably results from a failure of the mechanisms which normally maintain immunological unresponsiveness to FVIII. An increasingly large therapeutic armamentarium often enables bleeds to be treated effectively with coagulation factor concentrates (or occasionally, desmopressin) while an attempt is made to lower the concentration of the antibody by immunomodulation with intravenous immunoglobulin and immunosuppressive drugs. A consequence of the high cost of the coagulation factor concentrates and the not infrequent need to provide treatment over long periods of time is that such treatment often has serious resource implications.