Volume 11 (2024): Issue 1

Emicizumab in two patients with acquired haemophilia A – case report

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Milly Zhao
Thomas Kartika ORCID iD
Corey Witenko ORCID iD
Jessica Snead ORCID iD
Maria T DeSancho ORCID iD
Alana Ciolek ORCID iD

Abstract

Abstract

Patients with acquired haemophilia A (PwAHA) can present with severe bleeding and may require lengthy treatment with bypassing agents and immunosuppression. We present two cases of the implementation of emicizumab in PwAHA. The first patient, an 82-year-old man with rheumatoid arthritis (RA), presented with acquired haemophilia A (AHA) and spontaneous left tibia hematoma complicated by a persistent wound and infections. After a month of intermittent bleeding at the site and immunosuppression, the inhibitor level remained elevated and he was placed on emicizumab. While on therapy, debridement of the wound required activated factor VII therapy, which was complicated by a venous thromboembolism (VTE). He was successfully managed with anticoagulation while on emicizumab until his inhibitor level was undetectable. The second patient is a 62-year-old woman, also with RA and with a persistently positive dilute Russell viper venom time who presented with intracerebral haemorrhage (ICH) and was found to have AHA. After a period of time on bypassing agents, emicizumab was started due to a persistent inhibitor level and the ICH remained stable. She only required two doses initially, however, had a relapse with recurrent factor VIII inhibitor and received three additional doses without any complications. These cases highlight that emicizumab is a viable option in the care of PwAHA in challenging scenarios such as in the context of VTE and ICH.

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References

  • 1. 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.
  • 2. Swindle JP, Xu Y, Mu Y, Solari PG. Healthcare costs among patients with hemophilia A treated with factor replacement or bypassing agents. Curr Med Res Opin 2019; 35(8): 1433–40. doi: 10.1080/03007995.2019.1596437.
  • 3. Hemlibra (emicizumab-kxwh) [package insert] [Internet]. South San Francisco, CA: Genentech, Inc.; 2017. Available from https://www.accessdata.fda.gov/drugsatfda_docs/label/2017/761083s000lbl.pdf (accessed February 2024).
  • 4. Knoebl P, Thaler J, Jilma P, Quehenberger P, Gleixner K, Sperr WR. Emicizumab for the treatment of acquired hemophilia A. Blood 2021; 137(3): 410–9. doi: 10.1182/blood.2020006315.
  • 5. Pishko AM, Doshi BS. Acquired hemophilia A: Current guidance and experience from clinical practice. J Blood Med 2022; 13: 255–65. doi: 10.2147/JBM.S284804.
  • 6. Mohinani A, Patel S, Tan V, et al. Desmopressin as a hemostatic and blood sparing agent in bleeding disorders. Eur J Haematol 2023; 110(5): 470–9. doi: 10.1111/ejh.13930.
  • 7. Tiede A, Kemkes-Matthes B, Knöbl P. Should emicizumab be used in patients with acquired hemophilia A? J Thromb Haemost 2021; 19(3): 637–44. doi: 10.1111/jth.15208.
  • 8. Tiede A, Collins P, Knoebl P, et al. International recommendations on the diagnosis and treatment of acquired hemophilia A. Haematologica 2020; 105(7): 1791–801. doi: 10.3324/haematol.2019.230771.
  • 9. Oldenburg J, Mahlangu JN, Bujan W, et al. The effect of emicizumab prophylaxis on health-related outcomes in persons with haemophilia A with inhibitors: HAVEN 1 Study. Haemophilia 2019; 25(1): 33–44. doi: 10.1111/hae.13618.
  • 10. Mancuso ME, Mahlangu J, Sidonio R, et al. Health-related quality of life and caregiver burden of emicizumab in children with haemophilia A and factor VIII inhibitors-Results from the HAVEN 2 study. Haemophilia 2020; 26(6): 1009–18. doi: 10.1111/hae.14183.
  • 11. Mahlangu J, Oldenburg J, Paz-Priel I, et al. Emicizumab prophylaxis in patients who have hemophilia A without inhibitors. N Engl J Med 2018; 379(9): 811–22. doi: 10.1056/NEJMoa1803550.
  • 12. Pipe SW, Shima M, Lehle M, et al. Efficacy, safety, and pharmacokinetics of emicizumab prophylaxis given every 4 weeks in people with haemophilia A (HAVEN 4): a multicentre, open-label, non-randomised phase 3 study. Lancet Haematol 2019; 6(6): e295–305. doi: 10.1016/S2352-3026(19)30054-7.
  • 13. Levy GG, Asikanius E, Kuebler P, Benchikh El Fegoun S, Esbjerg S, Seremetis S. Safety analysis of rFVIIa with emicizumab dosing in congenital hemophilia A with inhibitors: Experience from the HAVEN clinical program. J Thromb Haemost 2019; 17(9): 1470–7. doi: 10.1111/jth.14491.
  • 14. Takeyama M, Nogami K, Matsumoto T, Noguchi-Sasaki M, Kitazawa T, Shima M. An anti-factor IXa/factor X bispecific antibody, emicizumab, improves ex vivo coagulant potentials in plasma from patients with acquired hemophilia A. J Thromb Haemost 2020; 18(4): 825–33. doi: 10.1111/jth.14746.
  • 15. Pasca S, Zanon E, Mannucci PM, Peyvandi F. Emicizumab in acquired hemophilia A: pros and cons of a new approach to the prevention and treatment of bleeding. Blood Transfus 2023; 21(6): 549–56. doi: 10.2450/2023.0247-22.
  • 16. Shima M, Amano K, Ogawa Y, et al. A prospective, multicenter, open-label phase III study of emicizumab prophylaxis in patients with acquired hemophilia A. J Thromb Haemost 2023; 21(3): 534–45. doi: 10.1016/j.jtha.2022.10.004.
  • 17. Hess KJ, Patel P, Joshi AM, Kotkiewicz A. Utilization of emicizumab in acquired factor VIII deficiency. Am J Case Rep 2020; 21: e922326. doi: 10.12659/AJCR.922326.
  • 18. Dane KE, Lindsley JP, Streiff MB, Moliterno AR, Khalid MK, Shanbhag S. Successful use of emicizumab in a patient with refractory acquired hemophilia A and acute coronary syndrome requiring percutaneous coronary intervention. Res Pract Thromb Haemost 2019; 3(3): 420–3. doi: 10.1002/rth2.12201.
  • 19. Chen SL, Ellsworth P, Kasthuri RS, Moll S, Ma AD, Key NS. Emicizumab reduces re-hospitalization for bleeding in acquired haemophilia A. Haemophilia 2021; 27(4): e585–8. doi: 10.1111/hae.14335.
  • 20. Möhnle P, Pekrul I, Spannagl M, Sturm A, Singh D, Dechant C. Emicizumab in the treatment of acquired haemophilia: a case report. Transfus Med Hemother 2019; 46(2): 121–3. doi: 10.1159/000497287.
  • 21. Yates SG, Webb CB, Sarode R, Ibrahim IF, Shen YMP. Utilization of emicizumab in acquired hemophilia A: a case report. Transfus Apher Sci 2022; 61(6): 103457. doi: 10.1016/j. transci.2022.103457.
  • 22. Escobar M, Aboshady I, Montanez N. Prophylactic potential of standard and modified emicizumab prophylaxis in 2 patients with acquired hemophilia: a case report [abstract]. Res Pract Thromb Haemost 2020; 4 (Suppl 1). Available from https://abstracts.isth.org/abstract/prophylactic-potential-of-standard-and-modified-emicizumab-prophylaxis-in-2-patients-with-acquired-hemophilia-a-case-report/ (accessed February 2024).
  • 23. Gelbenegger G, Traby L, Rahimi N, Knöbl P. Management of acquired haemophilia A in severe Covid-19: Haemostatic bridging with emicizumab to keep the balance between bleeding and thrombosis. Br J Clin Pharmacol 2023; 89(2): 908–13. doi: 10.1111/bcp.15598.
  • 24. Al-Banaa K, Alhillan A, Hawa F, et al. Emicizumab use in treatment of acquired hemophilia A: a case report. Am J Case Rep 2019; 20: 1046–8. doi: 10.12659/AJCR.916783.
  • 25. Latef TJ, Bhardwaj P, Bilal M. Refractory acquired haemophilia A in a patient with HIV treated with Emicizumab. Blood Coagul Fibrinolysis 2022; 33(2): 138–40. doi: 10.1097/MBC.0000000000001118.
  • 26. Flommersfeld S, Slonka J, Bieberle I, Stockschläder A, Kemkes-Matthes B, Sachs UJ. Successful control of bleeding with emicizumab in aquired haemophilia A: a case report. Poster I P01 Acquired Coagulation Disorders/Thrombocytopenias [abstract]. Hämostaseologie 2019; 39(S01). doi: 10.1055/s-0039-1680166.
  • 27. Hansenne A, Hermans C. Emicizumab in acquired haemophilia A: about two clinical cases and literature review. Ther Adv Hematol 2021; 12: 20406207211038193. doi: 10.1177/20406207211038193.
  • 28. Crossette-Thambiah C, Arachchillage D, Laffan M. Postpartum acquired haemophilia A in the COVID era – building the case for emicizumab? [abstract]. Available from https://abstracts.isth.org/abstract/post-partum-acquired-haemophilia-a-in-the-covid-era-building-the-case-for-emicizumab/ (accessed February 2024).
  • 29. Happaerts M, Vanassche T. Acquired hemophilia following COVID-19 vaccination: Case report and review of literature. Res Pract Thromb Haemost 2022; 6(6): e12785. doi: 10.1002/rth2.12785.
  • 30. Kruse-Jarres R, Peyvandi F, Oldenburg J, et al. Surgical outcomes in people with hemophilia A taking emicizumab prophylaxis: experience from the HAVEN 1-4 studies. Blood Adv 2022; 6(24): 6140–50. doi: 10.1182/bloodadvances.2022007458.

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Authors

  • Milly Zhao

    Department of Pharmacy, New York-Presbyterian/Weill Cornell Medical Center, New York, NY, USA

  • Thomas Kartika

    ORCID iD
    rml9011@nyp.org
    Division of Hematology and Medical Oncology, Department of Medicine, New York-Presbyterian/Weill Cornell Medical Center, New York, NY, USA

  • Corey Witenko

    ORCID iD
    Department of Pharmacy, New York-Presbyterian/Weill Cornell Medical Center, New York, NY, USA

  • Jessica Snead

    ORCID iD
    Department of Pharmacy, New York University Langone Health, Brooklyn, NY, USA

  • Maria T DeSancho

    ORCID iD
    Division of Hematology and Medical Oncology, Department of Medicine, New York-Presbyterian/Weill Cornell Medical Center, New York, NY, USA

  • Alana Ciolek

    ORCID iD
    Department of Pharmacy, New York-Presbyterian/Weill Cornell Medical Center, New York, NY, USA