Acquired hemophilia: from symptoms to diagnosis: 616.151.514-07

Abstract

Summary. Acquired hemophilia (АН) is one of the rarest autoimmune diseases, with a severe course and a high mortality rate. Its diagnosis and treatment are complicated by the lack of sufficient research. One of the most challenging aspects of this disease is the fact that the majority of specialists recognize it as a hereditary pathology that predominantly affects men. According to the literature, the prevalence of АН is similar in both men and women. It should be noted that the risk of developing the disease increases significantly with age. AH has a wide range of potential triggers. There is a unified therapy algorithm, but the choice of treatment strategies depends on the patient’s condition, age, and comorbidities. After treatment completion, monthly monitoring of the patient and his parameters are recommended. Thus, AH can occur in the practice of a physician of any specialty and has a very high risk of unfavorable outcome. Its study will enable medical practitioners to diagnose it at an earlier stage and identify the first signs of the disease, thus facilitating the selection of an appropriate course of treatment. The collected materials are intended to raise awareness among specialists in various fields.

For citation: Pavlova V. Yu., Kretz A.I., Turenko E.A. Acquired hemophilia: from symptoms to diagnosis. Tromboz, gemostaz i reologiya. 2024;(4):4–11.  (In Russ.).

References 

1. Fu P.‐A., Chen C.‐W., Hsu Y.‐T. et al. A case of acquired hemophilia A and bullous pemphigoid following SARS‐CoV‐2 mRNA vaccination. J Formos Med Assoc. 2022;121(9):1872–6. DOI: 10.1016/j. jfma.2022.02.017. 
2. García‐Chávez J., Majluf‐Cruz A. Acquired hemophilia. Gac Med Mex. 2020;156(1):67–77. DOI: 10.24875/GMM.19005469. 
3. Ershov V.I., Gadaev I.Yu., Budanova D.A. et al. Literature review and clinical observation of acquired idiopathic hemophilia with a new missense mutation in the factor VIII gene (His2026Arg). Terapevticheskij arhiv. 2018;90(7):118–22. (In Russ.). DOI: 10.26442/ terarkh2018907118‐122. 
4. Charlebois J., Rivard G.É., St‐Louis J. Management of acquired hemophilia A: Review of current evidence. Transfus Apher Sci. 2018;57(6):717–20. DOI: 10.1016/j.transci.2018.10.011. 
5. Kabaeva E.N., Tsvirko D.G., Talako T.M. Acquired hemophilia: diagnostics and treatment. Gematologiya. Transfuziologiya. Vostochnaya Evropa. 2021;7(3):328–37. (In Russ.). DOI: 10.34883/PI.2021.7.3.006. 
6. Kessler C.M., Knöbl P. Acquired haemophilia: an overview for clinical practice. Eur J Haematol. 2015;95 Suppl 81:36–44. DOI: 10.1111/ ejh.12689. 
7. Galstyan G.M., Nalbandyan S.A., Sabirov K.R. et al. Treatment tactics for a patient with acquired hemophilia: continuous infusion of recombinant activated coagulation factor VII and the inhibitor eradication. Gematologiya i transfuziologiya. 2022;67(2):282–94. (In Russ.). DOI: 10.35754/0234‐5730‐2022‐67‐2‐282‐294.  
8. Franchini M., Vaglio S., Marano G. et al. Acquired hemophilia A: a review of recent data and new therapeutic options. Hematology. 2017;22(9):514–20. DOI: 10.1080/10245332.2017.1319115.  
9. Prasolov N.V., Bulanov A.Yu., Shulutko E.M., Orel E.B. Acquired hemophilia A in elderly patients. Klinicheskaya gerontologiya. 2015;21(9–10):16–24. (In Russ.). 
10. Mingot‐Castellano M. E., Núñez R., Rodríguez‐Martorell F. J. Acquired haemophilia: Epidemiology, clinical presentation, diagnosis and treatment. Med Clin (Barc). 2017;148(7):314–22. (In English, Spanish). DOI: 10.1016/j.medcli.2016.11.030. 
11. Makita S., Aoki T., Watarai A. et al. Acquired hemophilia associated with autoimmune bullous diseases: a report of two cases and a review of the literature. Intern Med. 2013;52(7):807–10. DOI: 10.2169/internalmedicine.52.9317. 
12. Surenkov A.A., Orel E.B., Zozulya N.I., Dvirnyk V.N. Features of clinical and laboratory diagnosis of rare coagulopathy acquired hemophilia. Gematologiya i transfuziologiya. 2022;67(4):535–50. (In Russ.). DOI: 10.35754/0234‐5730‐2022‐67‐4‐535‐550. 
13. Regino C.A., Alvarez J.C., Mejía Buriticá L. et al. Idiopathic acquired hemophilia A, a rare cause of bleeding: a case report and literature review. Am J Case Rep. 2021;22:e929401. DOI: 10.12659/ AJCR.929401. 
14. Tiede A., Werwitzke S., Scharf R.E. Laboratory diagnosis of acquired hemophilia A: limitations, consequences, and challenges. Semin Thromb Hemost. 2014;40(7):803–11. DOI: 10.1055/ s‐0034‐1390004. 
15. Mulliez S. M., Vantilborgh A., Devreese K. M. Acquired hemophilia: a case report and review of the literature. Int J Lab Hematol. 2014;36(3):398–407. DOI: 10.1111/ijlh.12210. 
16. Constantinescu C., Jitaru C., Pasca S. et al. Unexplained hemorrhagic syndrome? Consider acquired hemophilia A or B. Blood Rev. 2022;53:100907. DOI: 10.1016/j.blre.2021.100907. 
17. Dolan G., Benson G., Bowyer A. et al. Principles of care for acquired hemophilia. Eur J Haematol. 2021;106(6):762–73. DOI: 10.1111/ ejh.13592. 
18. Barlamov P.N., Vasilieva E.R., Golubeva M.E. et al. Acquired inhibitory form of hemophilia. Klinicheskaya medicina. 2018;96(4):361–4. (In Russ.). DOI: 10.18821/0023‐2149‐2018‐96‐4‐361‐364. 
19. Zdziarska J., Musiał J. Acquired hemophilia A: an underdiagnosed, severe bleeding disorder. Pol Arch Med Wewn. 2014;124(4):200–6. DOI: 10.20452/pamw.2192. 
20. Windyga J., Baran B., Odnoczko E. et al. Treatment guidelines for acquired hemophilia A. Ginekol Pol. 2019;90(6):353–64. DOI: 10.5603/GP. 2019.0063. 
21. Esteves Pereira M., Bocksrucker C., Kremer Hovinga J.A. et al. Immunoadsorption for the treatment of acquired hemophilia: new observational data, systematic review, and metaanalysis. Transfus Med Rev. 2021;35(2):125–34. DOI: 10.1016/ j.tmrv.2021.01.001. 
22. Tiede A., Worster A. Lessons from a systematic literature review of the effectiveness of recombinant factor VIIa in acquired haemophilia. Ann Hematol. 2018;97(10):1889–901. DOI: 10.1007/ s00277‐018‐3372‐z.