Anticoagulation for Stroke Prevention after Restoration of Haemostasis with Emicizumab in Acquired Haemophilia A
  • Kadhim Al-Banaa
    Department of Hematology/Oncology, Oakland University William Beaumont School of Medicine, Royal Oak, MI, USA
  • Nicolas Gallastegui-Crestani
    Department of Medicine, Division of Hematology/Oncology, University of California San Diego, San Diego, CA, USA
  • Annette von Drygalski
    Department of Medicine, Division of Hematology/Oncology, University of California San Diego, San Diego, CA, USA; Department of Molecular Medicine, Scripps Research Institute, La Jolla, CA, USA

Keywords

Acquired haemophilia A, haemorrhage, emicizumab, anticoagulation failure, venous thromboembolism, atrial fibrillation

Abstract

Acquired haemophilia A (AHA) is a rare haemorrhagic disorder caused by the development of autoantibodies inhibiting factor VIII function. It predominantly affects the elderly, who are often burdened with a considerable number of comorbidities, and can result in life-threatening bleeding. The management of AHA consists of two aspects: inhibitor eradication with an immunomodulator and bleed control with a bypassing agent.

Here we present a case of AHA with a high titre inhibitor in a patient with extensive comorbidities and atrial fibrillation in whom inhibitor eradication could not be achieved within a few weeks using corticosteroids alone. Due to coronavirus disease (COVID)-19 restrictions and complications of care, emicizumab offered an effective and convenient therapy, not only sparing the need for continued and intensified inhibitor eradication, but also allowing anticoagulation for stroke prophylaxis.

VIEW THE ENTIRE ARTICLE

References

  • Franchini M, Lippi G. Acquired factor VIII inhibitors.Blood 2008;112(2):250–255.
  • Dolan G, Benson G, Bowyer A, Eichler H, Hermans C, Jiménez-Yuste V, et al. Principles of care for acquired hemophilia. Eur J Haematol 2021;106(6):762–773.
  • Tiede A, Collins P, Knoebl P, Teitel J, Kessler C, Shima M, et al. International recommendations on the diagnosis and treatment of acquired hemophilia A. Haematologica 2020;105(7):1791.
  • Collins P, Baudo F, Knoebl P, Lévesque H, Nemes L, Pellegrini F, et al. Immunosuppression for acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2). Blood 2012;120(1):47–55.
  • Oldenburg J, Mahlangu JN, Kim B, Schmitt C, Callaghan MU, Young G, et al. Emicizumab prophylaxis in hemophilia A with inhibitors. N Engl J Med 2017;377(9):809–818.
  • Mahlangu J, Oldenburg J, Paz-Priel I, Negrier C, Niggli M, Mancuso ME, et al. Emicizumab prophylaxis in patients who have hemophilia A without inhibitors. N Engl J Med 2018;379(9):811–822.
  • Pipe SW, Shima M, Lehle M, Shapiro A, Chebon S, Fukutake K, 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.
  • Al-Banaa K, Alhillan A, Hawa F, Mahmood R, Zaki A, El Abdallah M, et al. Emicizumab use in treatment of acquired hemophilia A: a case report. Am J Case Rep 2019;20:1046.
  • 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):420–423.
  • 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):121–123.
  • Knoebl P, Thaler J, Jilma P, Quehenberger P, Gleixner K, Sperr WR. Emicizumab for the treatment of acquired hemophilia A. Blood 2021;137(3):410–419.
  • Knöbl P. Prevention and management of bleeding episodes in patients with acquired hemophilia A. Drugs 2018;78(18):1861–1872.
  • Muto A, Yoshihashi K, Takeda M, Kitazawa T, Soeda T, Igawa T, et al. Anti-factor IXa/X bispecific antibody ACE910 prevents joint bleeds in a long-term primate model of acquired hemophilia A. Blood 2014;124(20):3165–3171.
  • Ferrière S, Peyron I, Christophe OD, Kawecki C, Casari C, et al. A hemophilia A mouse model for the in vivo assessment of emicizumab function. Blood 2020;136(6):740–748.
  • Lenting PJ, Denis CV, Christophe OD. Emicizumab, a bispecific antibody recognizing coagulation factors IX and X: how does it actually compare to factor VIII?. Blood 2017;130(23):2463–2468.
  • Oldenburg J, Mahlangu JN, Bujan W, Trask P, Callaghan MU, Young G, 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.
  • Available from www.emicizumabinfo.global/content/dam/hemlibra/global/en/congresses/eahad-2020/EAHAD%202020_Oral_Lee_Postmarket%20Safety%20Update.pdf [accessed 2 November, 2021].
  • Martin K, Key NS. How I treat patients with inherited bleeding disorders who need anticoagulant therapy. Blood 2016;128(2):178–184.
  • Chhabra M, Hii ZW, Rajendran J, Ponnudurai K, Fan BE. Venous thrombosis in acquired hemophilia: the complex management of competing pathologies. TH Open 2019;3(04):e325–330.
  • Weyand AC, Dorfman AL, Shavit JA, Pipe SW. Emicizumab prophylaxis to facilitate anticoagulant therapy for management of intra-atrial thrombosis in severe haemophilia with an inhibitor.Haemophilia 2019;25(3):e203–205.
  • Views: 394
    HTML downloads: 66
    PDF downloads: 141


    Published: 2021-11-11
    Issue: 2021: Vol 8 No 11 (view)


    How to cite:
    1.
    Al-Banaa K, Gallastegui-Crestani N, von Drygalski A. Anticoagulation for Stroke Prevention after Restoration of Haemostasis with Emicizumab in Acquired Haemophilia A . EJCRIM 2021;8 doi:10.12890/2021_002894.

    Most read articles by the same author(s)