The successful management of stroke in Evans syndrome by anticoagulation with warfarin, intravenous immunoglobulin (IVIG), and high-dose corticosteroid
  • Toshiaki Takahashi
    Department of Medicine, John A. Burns School of Medicine, University of Hawaii, Honolulu, USA
  • Kensuke Takaoka
    Department of Medicine, John A. Burns School of Medicine, University of Hawaii, Honolulu, USA
  • Kelsey Kwong
    Department of Medicine, John A. Burns School of Medicine, University of Hawaii, Honolulu, USA
  • Sharina Macapagal
    Department of Medicine, John A. Burns School of Medicine, University of Hawaii, Honolulu, USA
  • Manasawee Tanariyakul
    Department of Medicine, John A. Burns School of Medicine, University of Hawaii, Honolulu, USA
  • Chalothorn Wannaphut
    Department of Medicine, John A. Burns School of Medicine, University of Hawaii, Honolulu, USA
  • Yoshito Nishimura
    Department of Medicine, John A. Burns School of Medicine, University of Hawaii, Honolulu, USA

Keywords

Evans syndrome, coagulopathy, stroke of undetermined aetiology, stroke, AIHA

Abstract

Evans syndrome (ES) is a rare autoimmune disorder characterised by autoimmune haemolytic anaemia (AIHA), immune thrombocytopenia and autoimmune neutropenia. The precise pathogenesis of ES remains unclear, but it is believed to involve immune-mediated destruction of erythrocytes and platelets. Thrombotic complications, such as stroke, are critical yet largely unrecognised in ES. Here, we present a case of an 80-year-old male with ES who developed multiple strokes, emphasising the complex management challenges associated with this condition. The patient, known for stage IIB lung adenocarcinoma, presented with right-sided weakness and was diagnosed with a stroke of undetermined aetiology. He was started on warfarin for secondary prevention alongside intravenous immunoglobulin (IVIG) and corticosteroids for ES. Stroke in ES is rarely reported, and the optimal management remains inconclusive due to its rarity. The patient’s management was guided by existing guidelines for stroke prevention and anticoagulation in the setting of antiphospholipid syndrome. While anticoagulants are recommended for venous thromboembolism prophylaxis in AIHA, there are no clear guidelines for stroke prevention in ES. This case underscores the necessity of individualised treatment approaches and highlights the gaps in evidence regarding stroke management in ES. Future research is essential to determine the optimal management of stroke in this complex clinical scenario.

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References

  • Fattizzo B, Michel M, Giannotta JA, Hansen DL, Arguello M, Sutto E, et al. Evans syndrome in adults: an observational multicenter study. Blood Adv 221;5:5468–5478.
  • Evans RS, Takahashi K, Duane RT, Payne R, Liu C. Primary thrombocytopenic purpura and acquired hemolytic anemia: evidence for a common etiology. AMA Arch Intern Med 1951;87:48–65.
  • Hansen DL, Möller S, Andersen K, Gaist D, Frederiksen H. Evans syndrome in adults – incidence, prevalence, and survival in a nationwide cohort. Am J Hematol 2019;94:1081–1090.
  • Jaime-Pérez JC, Aguilar-Calderón PE, Salazar-Cavazos L, Gómez-Almaguer D. Evans syndrome: clinical perspectives, biological insights and treatment modalities. J Blood Med 2018;9:171–184.
  • Norton A, Roberts I. Management of Evans syndrome. Br J Haematol 2006;132:125–137.
  • Hendrick AM. Auto-immune haemolytic anaemia – a high-risk disorder for thromboembolism? Hematology 2003;8:53–56.
  • Shin JW, Nah H-W, Song H-J. Recurrent and refractory ischemic stroke complicating Evans syndrome. Clin Surg 2021;6:3066.
  • Patel G, Limgala P, Patel P, Makar M, Barooah J, Pir MS, et al. FIT Clinical Decision Making. Embolic stroke in a patient with Evans syndrome receiving rituximab therapy. J Am Coll Cardiol 2019;73:2817.
  • Michel M, Chanet V, Dechartres A, Morin AS, Piette JC, Cirasino L, et al. The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases. Blood 2009;114:3167–3172.
  • Adams HP, Bendixen BH, Kappelle LJ, Biller J, Love BB, Gordon DL, et al. Classification of subtype of acute ischemic stroke. Definitions for use in a multicenter clinical trial. TOAST: Trial of ORG 10172 in Acute Stroke Treatment. Stroke 1993;24:35–41.
  • Tektonidou MG, Andreoli L, Limper M, Amoura Z, Cervera R, Costedoat-Chalumeau N, et al. EULAR recommendations for the management of antiphospholipid syndrome in adults. Ann Rheum Dis 2019;78:1296–1304.
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    Published: 2024-06-28
    Issue: 2024: Vol 11 No 7 (view)


    How to cite:
    1.
    Takahashi T, Takaoka K, Kwong K, Macapagal S, Tanariyakul M, Wannaphut C, Nishimura Y. The successful management of stroke in Evans syndrome by anticoagulation with warfarin, intravenous immunoglobulin (IVIG), and high-dose corticosteroid. EJCRIM 2024;11 doi:10.12890/2024_004592.