Acquired hemophilia A: when an overlooked autoimmune disorder causes significant bleeding

Abstract

ABSTRACTIntroduction: Acquired hemophilia A (AHA) is a rare disease caused by the presence of autoantibodies directed against factor VIII (FVIII) resulting in spontaneous bleeding. AHA is associated with high risk of mortality from bleeding, treatment complications, and the presence of underlying diseases. Because of its rarity, AHA diagnosis can be challenging and sometimes missed in the emergency setting. Management consists of bleeding control and prevention, inhibitor eradication using immunosuppressive therapy, as well as treatment of the underlying disease.Areas covered: An overview of AHA including epidemiology, pathophysiology, clinical presentation, workup, and management; as well as a review of the literature in order to better understand the disease.Expert opinion: A prompt recognition of AHA is mandatory for the initiation of early and aggressive treatment. Optimal management of AHA necessitates a multidisciplinary approach in collaboration between hematologists and physicians from various specialties. Future studies are needed to identify prognostic factors for remission and survival in patients with AHA. Clinicians who treat patients with AHA are encouraged to actively contribute to the available registries. International associations and societies are also invited to assist in the establishment of hemophilia centers and networks worldwide for the standardization of clinical practice when dealing with patients with AHA.