Current trends and advances in the management of acquired Hemophilia A

Authors: Zuner, E; Désage, S; Rezigue, H; Dargaud, Y; Lienhart, A; Nougier, C

Affiliations: French Reference Center for Hemophilia, Clinical Hemostasis Unit, Hôpital Cardiologique Louis Pradel, Hospices Civils de Lyon, France. French Reference Center for Hemophilia, Clinical Hemostasis Unit, Hôpital Cardiologique Louis Pradel, Hospices Civils de Lyon, France. Hemostasis Laboratory, Centre de Biologie et de Pathologie Est, Hospices Civils de Lyon, France ; UR4609 Hemosatsis & Thrombosis Research Unit, Faculté de Médecine Lyon Est, Université Claude Bernard Lyon 1, France. Hospices Civils de Lyon, France ; UR4609 Hemosatsis & Thrombosis Research Unit, Faculté de Médecine Lyon Est, Université Claude Bernard Lyon 1, France.

Publication: Blood reviews; 2025. 101320

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 autoantibodies 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.