Next-generation treatment of acquired hemophilia A

Abstract

Introduction: Acquired hemophilia A (AHA) is a rare autoimmune disorder caused by factor VIII (FVIII) autoantibodies that interfere with FVIII coagulant function, predisposing affected patient to an often severe bleeding diathesis. The management of AHA is primarily directed toward the eradication of the autoantibody using immunosuppressive agents (i.e., steroids alone or in association with cyclophosphamide). Hemostatic treatment to control severe bleeding is based on the use of bypassing agents (i.e., activated prothrombin complex concentrate and recombinant activated factor VII).Areas covered: After an overview of the main clinical, diagnostic and therapeutic aspects of AHA, this review focuses on the investigational agents aimed at improving the control of bleeding in AHA patients, which represents the most important challenge among physicians in this clinical setting.Expert opinion: Among the promising new hemostatic drugs, recombinant porcine FVIII has been evaluated and recently approved for AHA treatment in the USA by FDA and provides an additional option for successfully controlling severe bleeding episodes in patients with FVIII autoantibodies.