Acquired hemophilia A: current approaches to treatment and clinical cases | Ukrainian Medical Journal

Classical hemophilia is an X-linked disease of the hemostasis system characterized by decreased or impaired synthesis of coagulation factors. Most doctors believe that hemophilia is a hereditary pathology, but in clinical practice, in addition to the hereditary form, there is acquired hemophilia, which is an extremely rare pathology, based on the production of autoantibodies that inactivate coagulation factors. The article presents a literature review on key aspects of the management of patients with hemophilia, focusing on the problem of acquired hemophilia A, as well as our own clinical observations of successful treatment of patients with inhibitors to coagulation factors.

References

1. Stasishin O.V. (2020) Hereditary disorders in the hemostasis system: focus on current diagnostic possibilities. UMJ, 6(1)(140). www.umj.com.ua/article/195042 (In Ukr.).
2. Knoebl P., Marco P., Baudo F. et al. (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.
3. Ewenstein B.M., Putnam K.G., Bohn R.L. (2002) Nonhemophilic inhibitors of coagulation. In: Kitchens CS, Alving BM, Kessler CM, editors. Consultative hemostasis and thrombosis. Philadelphia, PA: WB Saunders, p. 75–90.
4. Huth-Kühne A., Baudo F., Collins P. et al. (2009) International recommendations on the diagnosis and treatment of patients with acquired haemophilia. Haematologica, 94(4): 566–575.
5. Barlamov P.N., Shchekotov V.V.1, Shutylev A.A. et al. (2015) Postpartum acquired hemophilia. Klin. Med., 93(10): 67–71. (in Rus.).
6. Khair K., Chalmers E., Flannery T. et al. (2021) Expert opinion on the UK standard of care for haemophilia patients with inhibitors: a modified Delphi consensus study. Ther. Adv. Hematol., 12: 20406207211007058. r 30. делфи
7. Ljung R., Auerswald G., Benson G. et al. (2019) Inhibitors in haemophilia A and B: Management of bleeds, inhibitor eradication and strategies for difficult-to-treat patients. Eur. J. Haematol. 102(2): 111–122. doi:10.1111/ejh.13193.
8. Tieu P., Chan A., Matino D. (2020) Molecular Mechanisms of Inhibitor Development in Hemophilia. Mediterr. J. Hematol. Infect. Dis., 12(1): e2020001. doi:10.4084/MJHID.2020.001
9. Tretyakova O.S. (2012) Hemophilia in children: etiopathogenesis, clinical manifestations, diagnostic approaches. Dit. Lik., 3–4 (16–17): 26–35. (In Rus.).
10. Sakurai Y., Takeda T. (2014) Acquired hemophilia A: a frequently overlooked autoimmune hemorrhagic disorder. J. Immunol. Res., 2014: 320674. doi: 10.1155/2014/320674.
11. Kreuz W., Gill J.C., Rothschild C. et al. (2005) Full-length sucrose-formulated recombinant factor VIII for treatment of previously untreated or minimally treated young children with severe haemophilia A: results of an international clinical investigation. Thromb. Haemost., 93(3): 457–467. doi: 10.1160/TH03-10-0643.
12. Dimichele D., Négrier C. (2006) A retrospective postlicensure survey of FEIBA efficacy and safety. Haemophilia, 12(4): 352–362.
13. Wessel L.M., Scholz S., Rüsch M. (2001) Characteristic pattern and management of intraarticular knee lesions in different pediatric age groups. J. Pediatr. Orthop., 21(1): 14–19. doi: 10.1097/00004694-200101000-00005.
14. Funk M.B., Schmidt H., Becker S. et al. (2002) Modified magnetic resonance imaging score compared with orthopaedic and radiological scores for the evaluation of haemophilic arthropathy. Haemophilia, 8(2): 98–103. doi: 10.1046/j.1365–2516.2002.00585.x.
15. Averyanov E.V. (2015) Changes in the functional capacity of the extremities in patients with hemophilia with posthemorrhagic arthropathy. Hematology and blood transfusion: Collection of scientific works, 38: 345–346. (in Ukr.).
16. Khits A. (2020) Modern approaches to the treatment of patients with hemophilia: focus on the inhibitory form. UMJ, 5 (139). www.umj.com.ua/article/189488. (In Ukr.).
17. Collins, P., Baudo, F., Huth-Kühne A. et al. (2010) Consensus recommendations for the diagnosis and treatment of acquired hemophilia A. BMC Res. Notes, 3: 161. doi.org/10.1186/1756-0500-3-161.
18. Yousphi A.S., Bakhtiar A., Cheema M.A. et al. (2019) Acquired Hemophilia A: A Rare but Potentially Fatal Bleeding Disorder. Cureus, 11(8): e5442. doi:10.7759/cureus.5442.
19. Shander A., Walsh C.E., Cromwell C. (2011) Acquired hemophilia: a rare but life-threatening potential cause of bleeding in the intensive care unit. Intens. Care Med., 37(8): 1240–1249. doi: 10.1007/s00134-011-2258-5.
20. Franchini M., Mannucci P.M. (2013) Acquired haemophilia A: a 2013 update. Thromb. Haemost., 110(6): 1114–1120. doi: 10.1160/TH13-05-0363.