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


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


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.



  • 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 [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.
  • Published: 2021-11-11

    Issue: 2021: Vol 8 No 11 (view)

    Section: Articles

    How to cite:
    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.

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