Combined Upfront Immunosuppression for Acquired Hemophilia A

Acquired haemophilia A (AHA) is an extremely rare autoimmune disorder caused by autoantibodies to coagulation factor VIII and characterized by life-threatening bleeding within soft tissues and vital organs. Supportive therapy for bleed control and immunosuppressive therapy remain the backbone of treatment, but there is no consensus about the best immunosuppressive therapy. Most regimens include upfront steroids followed by other immunosuppressive agents (e.g., rituximab, cyclophosphamide, cyclosporine) when steroids fail.

In an attempt to reduce steroid exposure and toxicity, investigators in Hungary instituted an upfront, combined, pulse-dosed immunosuppressive treatment protocol in 2009. Now, they report outcomes for the first 32 patients …