[Acquired haemophilia due to autoantibodies against factor VIII]

The acquired haemophilia is caused by formation of autoantibodies which are most frequently directed against factor VIII (FVIII). This disease associated with considerable morbidity as well as mortality occurs preferentially in the elderly. The antibodies lead to a loss of FVIII activity resulting in bleeds especially of soft or subcutaneous tissue or in muscles.

aPTT prolongation and the reduction of the factor activity are of diagnostic importance. However, with respect to bleeding tendency basal activity and inhibitor titre are less meaningful than in inherited haemophilia complicated by inhibitor formation. Therapy is subdivided into treatment of acute bleeds and long-term eradication of the inhibitor. Administration of recombinant FVIIa, high dosed FVIII or activated prothrombin complex concentrates represent approved treatment options of bleeding episodes. Immunosuppressive agents occasionally combined with FVIII administration or immunoadsorption may lead to long-term inhibitor eradication. As CD4(+) T cells as well as anti-idiotypic antibodies appear to be increasingly pathogenetically meaningful new immunomodulating agents might improve the treatment of acquired haemophilia.