Current trends and advances in the management of acquired Hemophilia A

Abstract

Acquired hemophilia A (AHA), a rare and life-threatening bleeding disorder of adults, caused by anti-factor VIII (FVIII) autoantibodies, is often underestimated, particularly in patients receiving antithrombotic therapy. AHA is often associated with autoimmune disease, cancer, infection or pregnancy. AHA, characterized by severe spontaneous bleeding, particularly in muscles and subcutaneous tissues, requires a timely and accurate diagnosis. Delayed diagnosis worsens the prognosis and increases the risk of complications. Disease confirmation requires identification of FVIII deficiency and anti-FVIII antibodies. Treatment focuses on managing acute bleeding episodes, addressing the underlying condition and eradicating auto-antibodies through immunosuppressive therapy. Bypassing agents are used for treatment, but promising new therapeutic options such as emicizumab are under evaluation. AHA remains a serious condition with high mortality from bleeding complications and adverse effects of immunosuppression. This review provides a comprehensive overview of current knowledge on AHA, including epidemiology, pathophysiology, diagnosis, prognostic factors and therapeutic strategies.

Keywords: Acquired hemophilia A; Anti-FVIII autoantibodies; Bleeding; Bypassing agents; Emicizumab; Immunosuppressive therapy.