ACQUIRED HEMOPHILIA A: CURRENT CONCEPTS IN EPIDEMIOLOGY, PATHOGENESIS, DIAGNOSIS, AND MANAGEMENT IN THE EMICIZUMAB ERA
DOI:
https://doi.org/10.31435/ijitss.3(51).2026.6483Keywords:
Acquired Hemophilia A, Factor VIII Inhibitor, Autoimmune Bleeding Disorder, Emicizumab, Immunosuppression, Bypassing AgentsAbstract
Acquired hemophilia A (AHA) is a rare autoimmune bleeding disorder caused by neutralizing autoantibodies against factor VIII, most often affecting older adults without a prior bleeding history. This narrative review summarizes current knowledge on its epidemiology, pathogenesis, diagnosis, and management, with special attention to emicizumab. A selective PubMed/MEDLINE search of English-language literature published from 2005 to 2025 was performed, prioritizing international guidelines, reviews, registry studies, and clinically relevant cohort reports. AHA typically presents as a hematologic emergency with soft-tissue and mucosal bleeding, isolated prolongation of activated partial thromboplastin time, low factor VIII activity, and a detectable factor VIII inhibitor. Management requires two parallel goals: rapid bleeding control with bypassing agents or recombinant porcine factor VIII, and inhibitor eradication using individualized immunosuppressive therapy. Although remission rates of 70–80% are achievable, mortality remains significant because of both severe bleeding and treatment-related toxicity in elderly, multimorbid patients. Emerging evidence suggests that emicizumab may reduce bleeding burden, limit exposure to intensive bypass therapy, and support safer treatment strategies in selected cases. Earlier recognition, standardized diagnostic pathways, and careful integration of emicizumab may improve outcomes in AHA.
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Copyright (c) 2026 Mateusz Winkler, Julia Bezak, Aleksandra Arczewska, Michał Zaborowski, Klaudia Purgał-Zaborowska, Klaudia Michałowska, Oliwier Kolanowski, Olga Turzańska, Diana Marcinkowska
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