Acquired inhibitory body hemophilia

Although autoantibodies against factor VIII or factor IX are a rare phenome non, they are associated with a high risk of bleeding and high mortality. T he condition, termed acquired haemophilia, occurs equally in both sexes and is most frequent in higher age groups. Patients typically present with sev ere bleedings in muscles and skin. In contrast to patients with congenital haemophilia and inhibitors,joint bleedings are very rare. In approximately half of all cases an associated disease state can be identified as the caus e of autoantibody formation. An immediate and comprehensive diagnosis is es sential for a rapid initiation of therapy. Equally important are a careful diagnostic differentiation between congenital and acquired factor deficienc ies and the exclusion of non-specific inhibitors, which increase the occurr ence of thrombolic events. The inhibitor titre, quantified using the Bethes da assay, is an important criterion for selecting the appropriate therapy. A wide range of treatment options is now available for the management of bl eedings in patients with acquired haemophilia, namely porcine factor VIII, recombinant factor VIIa, prothrombin complex concentrates, and immunoadsorp tions. in addition, immunosuppressive therapies a re used to achieve perman ent reduction or elimination of inhibitors.