Acquired hemophilia A: an illustrated review based on the French National Guidelines

Abstract

Acquired hemophilia A is a rare but potentially life-threatening autoimmune bleeding disorder caused by the sudden development of neutralizing autoantibodies against factor VIII (FVIII), predominantly affecting older adults. Because of the risk of severe bleeding and excess mortality, prompt hospitalization and immediate management are essential to avoid diagnostic and therapeutic delays. Diagnosis relies on the association of an isolated prolonged activated partial thromboplastin time, reduced FVIII activity, and detection of a FVIII inhibitor quantified using the Bethesda or Nijmegen assay. Management has 2 complementary objectives. First, hemostatic therapy combines preventive measures with treatment of acute bleeding episodes, using bypassing agents (recombinant activated FVIII or activated prothrombin complex concentrate) or recombinant porcine factor VIII as first-line options. More recently, prophylaxis with emicizumab has emerged as a promising strategy to reduce recurrent bleeding, with early clinical data suggesting favorable efficacy and safety. Second, inhibitor eradication relies on immunosuppressive therapy, typically corticosteroids alone or in combination with agents such as cyclophosphamide or rituximab, guided by baseline FVIII activity and inhibitor titer. Close and prolonged follow-up is mandatory, combining clinical assessment of bleeding control, recurrence risk, and treatment toxicity, with laboratory monitoring of hemoglobin, factor VIII activity, von Willebrand factor levels, and inhibitor titers. Surveillance should be maintained for at least 2 years after complete remission.

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BibTeX RIS

BibTeX

@article{guillet2026acquired,
  title = {{Acquired hemophilia A: an illustrated review based on the French National Guidelines}},
  author = {Benoit Guillet and Roseline d'Oiron and Sébastien Lacroix-Desmazes and Valérie Chamouard and Achille Aouba and Sabine Castet and Ygal Benhamou and Christophe Nougier and Nicolas Noel and Yohann Repessé and Julie Graveleau and Marc Trossaert and Hervé Lévesque and Yesim Dargaud},
  journal = {Research and Practice in Thrombosis and Haemostasis},
  year = {2026},
  month = jul,
  doi = {10.1016/j.rpth.2026.106820},
  url = {https://hal.science/hal-05752786v1},
  note = {CC BY 4.0},
}

RIS

TY  - JOUR
TI  - Acquired hemophilia A: an illustrated review based on the French National Guidelines
AU  - Benoit Guillet
AU  - Roseline d'Oiron
AU  - Sébastien Lacroix-Desmazes
AU  - Valérie Chamouard
AU  - Achille Aouba
AU  - Sabine Castet
AU  - Ygal Benhamou
AU  - Christophe Nougier
AU  - Nicolas Noel
AU  - Yohann Repessé
AU  - Julie Graveleau
AU  - Marc Trossaert
AU  - Hervé Lévesque
AU  - Yesim Dargaud
PY  - 2026
DA  - 2026/07/27
JO  - Research and Practice in Thrombosis and Haemostasis
DO  - 10.1016/j.rpth.2026.106820
UR  - https://hal.science/hal-05752786v1
AB  - Acquired hemophilia A is a rare but potentially life-threatening autoimmune bleeding disorder caused by the sudden development of neutralizing autoantibodies against factor VIII (FVIII), predominantly affecting older adults. Because of the risk of severe bleeding and excess mortality, prompt hospitalization and immediate management are essential to avoid diagnostic and therapeutic delays. Diagnosis relies on the association of an isolated prolonged activated partial thromboplastin time, reduced FVIII activity, and detection of a FVIII inhibitor quantified using the Bethesda or Nijmegen assay. Management has 2 complementary objectives. First, hemostatic therapy combines preventive measures with treatment of acute bleeding episodes, using bypassing agents (recombinant activated FVIII or activated prothrombin complex concentrate) or recombinant porcine factor VIII as first-line options. More recently, prophylaxis with emicizumab has emerged as a promising strategy to reduce recurrent bleeding, with early clinical data suggesting favorable efficacy and safety. Second, inhibitor eradication relies on immunosuppressive therapy, typically corticosteroids alone or in combination with agents such as cyclophosphamide or rituximab, guided by baseline FVIII activity and inhibitor titer. Close and prolonged follow-up is mandatory, combining clinical assessment of bleeding control, recurrence risk, and treatment toxicity, with laboratory monitoring of hemoglobin, factor VIII activity, von Willebrand factor levels, and inhibitor titers. Surveillance should be maintained for at least 2 years after complete remission.
SN  - 2475-0379
SN  - 2475-0379
ER  -

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