Immunotherapy of acquired hemophilia A

Acquired hemophilia A (AHA) is an autoimmune disorder characterized by the formation of autoantibodies that neutralize the function of coagulation factor VIII. Immunosuppressive therapy (IST) with glucocorticoids, cyclophosphamide, rituximab, or combinations thereof is the standard of care to suppress autoantibody formation and induce remission of AHA. About 80% of patients achieve remission over the course of a few weeks to several months. However, patients with AHA are often elderly and frail and have adverse events from IST. Therefore, guidelines suggest an individualized approach using caution in elderly and frail patients. Prophylaxis with emicizumab may reduce the need for early and aggressive IST in the future.