Acquired haemophilia: diagnosis and therapeutic management.

Purpose. - Acquired haemophilia is a rare disease, occurring most frequentl y in elderly patients, caused by the development of autoantibodies against factor VIII. Current Knowledge and key points. - The disease is characterised by spontan eous haemorrhagic complications which can be fatal in 15-20% of the patient s. However spontaneous remission is possible and in fact natural evolution and aetiology are still partly unknown. Acquired haemophilia may arise in a ssociation with auto-immune diseases, lymphoproliferative malignancy, pregn ancy and also as a drug reaction. The alms of the treatment are first to tr eat the bleeding which is the most common cause of morbidity and mortality, and second to eliminate the inhibitor by immunosuppression. However no con sensus exists for these two parts of the treatment. Bleeding may be control led by prothrombin complex concentrates, recombinant factor VIIa or porcine factor VIII. The inhibitor is abolished in up 70% of patients using predni sone and cyclophosphamide. Other combinations of prednisone with azathiopri ne or with cyclophosphamide and vincristine or the use of high-dose immunog lobulin or double-filtration plasmapheresis have also proven effective in s ome patients. Future and projects. - The rare occurrence of the disease, the associated w ith various diseases, and lack of consensus about treatment, require multic entric prospective studies. (C) 2001 Editions scientifiques et medicales El sevier SAS.