Acquired hemophilia A: key principles of diagnosis and management

May 4, 2022
Specialities :

Today, the problem of acquired hemophilia A remains very relevant with a relatively low level of awareness of doctors on this topic. Acquired hemophilia A is a rare, life-threatening, and often fatal coagulation disorder. The article presents algorithms for diagnosis and basic principles of treatment of this disease. A clinical case of acquired hemophilia in a woman in labor with the development of uterine bleeding, features of disease diagnosis and treatment tactics are presented.


  • 1. Collins P., Baudo F., Huth-Kühne A. et al. (2010) Consensus recommendations for the diagnosis and treatment of acquired hemophilia A. BMC Research Notes, 3: 161.
  • 2. Kessler C.M., Knöbl P. (2015) Acquired haemophilia: an overview for clinical practice. Eur. J. Haematol., 95 Suppl. 81: 36–44. doi: 10.1111/ejh.12689.
  • 3. Sakurai Y., Takeda T. (2014) Acquired hemophilia A: a frequently overlooked autoimmune hemorrhagic disorder. J. Immunol. Res., 2014: 320674. doi: 10.1155/2014/320674.
  • 4. Knoebl P., Marco P., Baudo F. et al.; EACH2 Registry Contributors (2012) Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2). J. Thromb. Haemost., 10(4): 622–631. doi: 10.1111/j.1538-7836.2012.04654.x.
  • 5. Green D., Lechner K. (1981) A survey of 215 non-hemophilic patients with inhibitors to Factor VIII. Thromb. Haemost., 45(3): 200–203.
  • 6. Zeng Y., Zhou R., Duan X. et al. (2014) Interventions for treating acute bleeding episodes in people with acquired hemophilia A. Cochrane Database Syst. Rev., 2014(8): CD010761. doi: 10.1002/14651858.CD010761.pub2.
  • 7. Coppola A., Favaloro E.J., Tufano A. et al. (2012) Acquired inhibitors of coagulation factors: part I-acquired hemophilia A. Semin. Thromb. Hemost., 38(5): 433–446. doi: 10.1055/s-0032-1315757.
  • 8. Kruse-Jarres R., Kempton Ch.L., Baudo F. et al. (2017) Acquired hemophilia A: Updated review of evidence and treatment guidance. Am. J. Hematol., 92(7): 695–705. doi: 10.1002/ajh.24777.
  • 9. Qian L., Ge H., Hu P. et al. (2019) Pregnancy-related acquired hemophilia A initially manifesting as pleural hemorrhage. Medicine (Baltimore), 98(3): e14119.
  • 10. Webert K.E. (2012) Acquired hemophilia A. Semin. Thromb. Hemost., 38(7): 735–741. doi: 10.1055/s-0032-1326779.
  • 11. Pardos-Gea J., Fernández-Díaz N., Parra R. et al. (2018) Diagnostic delay in acquired haemophilia: Analysis of causes and consequences in a 20-year Spanish cohort. Haemophilia, 24(3): e163–e166. doi: 10.1111/hae.13499.
  • 12. Tiede A., Collins P., Knoebl P. et al. (2020) International recommendations on the diagnosis and treatment of acquired hemophilia A. Haematologica, 105(7): 1791–1801. doi: 10.3324/haematol.2019.230771.
  • 13. Goudemand J., Rothschild Ch., Demiguel V. et al.; FVIII-LFB and Recombinant FVIII study groups (2006) Influence of the type of factor VIII concentrate on the incidence of factor VIII inhibitors in previously untreated patients with severe hemophilia A. Blood, 107(1): 46–51. doi: 10.1182/blood-2005-04-1371.
  • 14. Sallah S. (2004) Treatment of acquired haemophilia with factor eight inhibitor bypassing activity. Haemophilia, 10(2): 169–173. doi: 10.1046/j.1365-2516.2003.00856.x.
  • 15. Baudo F., Collins P., Huth-Kühne A. et al.; EACH2 registry contributors (2012) Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry. Blood, 120(1): 39–46. doi: 10.1182/blood-2012-02-408930.
  • 16. Borg J.Y., Négrier C., Durieu I. et al.; FEIBHAC Study Group (2015) FEIBA in the treatment of acquired haemophilia A: results from the prospective multicentre French ‘FEIBA dans l’hémophilie A acquise’ (FEIBHAC) registry. Haemophilia, 21(3): 330–337. doi: 10.1111/hae.12574.