Therapy of Acquired Hemophilia - Immunoadsorption and Rituximab Treatment for Immunosuppression and Substitution of Coagulation Factors

Abstract

Autoantibodies to coagulation factors may occur in hemophiliacs as adverse events following substitution therapy. Especially perioperative management of hemophilia patients may be complicated by inhibitors, and in many patients bleeding complications become life threatening. Treatment with bypassing agents of the coagulation cascade like high-dose substitution of FEIBA or rFVIIa is a useful management of acute bleeding complications, but sustained elimination of inhibitors must be the therapeutic goal in inhibitor patients. In children with inhibitors treatment according to the Malmo protocol is the gold standard [1]. In adults, however, this protocol seems to be less effective and complete B-cell suppression might be useful to induce immunotolerance. Therefore, we developed a new protocol combining immunomodulation by steroids, cytotoxic treatment and immunoadsorption with monoclonal anti-CD 20 antibody Rituximab. Data of the first 2 consecutive patients are discussed.