Acquired haemophilia:: diagnosis and therapeutic management.

Purpose. - Acquired haemophilia is a rare disease, occurring most frequently in elderly patients, caused by the development of autoantibodies against factor VIII. Current Knowledge and key points. - The disease is characterised by spontaneous haemorrhagic complications which can be fatal in 15-20% of the patients. However spontaneous remission is possible and in fact natural evolution and aetiology are still partly unknown. Acquired haemophilia may arise in association with auto-immune diseases, lymphoproliferative malignancy, pregnancy and also as a drug reaction. The alms of the treatment are first to treat the bleeding which is the most common cause of morbidity and mortality, and second to eliminate the inhibitor by immunosuppression. However no consensus exists for these two parts of the treatment. Bleeding may be controlled by prothrombin complex concentrates, recombinant factor VIIa or porcine factor VIII. The inhibitor is abolished in up 70% of patients using prednisone and cyclophosphamide. Other combinations of prednisone with azathioprine or with cyclophosphamide and vincristine or the use of high-dose immunoglobulin or double-filtration plasmapheresis have also proven effective in some patients. Future and projects. - The rare occurrence of the disease, the associated with various diseases, and lack of consensus about treatment, require multicentric prospective studies. (C) 2001 Editions scientifiques et medicales Elsevier SAS.