Rare Case of Secondary Evans Syndrome in Setting of Triple Positive Anti-Phospholipid Antibodies

Anahat Kaur MD¹, Bhanujit Dwivedi MBBS^2, , Tejasvi Dwivedi MBBS³, Rubina Sharma MD⁴ and Angad Singh MD⁵

¹Department of Hematology and Oncology, Albert Einstein College of Medicine, Jacobi Medical Center, Bronx, NY, USA

²Maulana Azad Medical College, New Delhi, India

³All India Institute of Medical Sciences, Raipur, India

⁴PGIMER, Chandigarh, India

⁵HCA Midwest Healthcare, Kansas City, KS, USA

Cite this paper

Anahat Kaur MD, Bhanujit Dwivedi MBBS, Tejasvi Dwivedi MBBS, Rubina Sharma MD and Angad Singh MD. Rare Case of Secondary Evans Syndrome in Setting of Triple Positive Anti-Phospholipid Antibodies. American Journal of Medical Case Reports. 2023; 11(7):123-125. doi: 10.12691/AJMCR-11-7-3

Abstract

Evans syndrome is a rare autoimmune disorder characterized by the simultaneous or sequential presence of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). Secondary Evans syndrome refers to cases in which these autoimmune manifestations occur in the setting of an underlying condition or trigger. We report the case of a 36 year old male with no significant past medical history who presented with symptoms of easy bruising, and mucocutaneous bleeding. Laboratory investigations revealed severe thrombocytopenia and hemolytic anemia, consistent with the diagnosis of Evans syndrome. Further evaluation demonstrated the presence of triple positive anti-phospholipid antibodies, including lupus anticoagulant, anticardiolipin antibodies, and anti-beta-2 glycoprotein antibodies. Patient was initially treated with steroids and IVIG but had refractory ITP for several weeks requiring rituximab (four weekly doses) and fostamatinib before eventual improvement and stabilization of platelet count. Given the rarity of secondary Evans syndrome with positive anti-phospholipid antibodies, there is a lack of robust evidence-based treatment guidelines. Our case highlights the complexity and challenges encountered in managing this condition.

Keywords

Secondary Evans, Anti-phospholipid antibodies, Refractory ITP

Copyright

This work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

References

[1]	Giannakopoulos B, Krilis SA. The pathogenesis of the antiphospholipid syndrome. N Engl J Med. 2013;368(11):1033-1044.
[2]	Cervera R, Piette JC, Font J, et al. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum. 2002;46(4):1019-1027.
[3]	Patel BP, Jakob J. A Rare Case of Simultaneous Evans Syndrome and Primary Antiphospholipid Syndrome. Cureus. 2020 Feb 1;12(2):e6845.
[4]	Al-Samkari H, Kuter DJ. Immune Thrombocytopenia in Adults: Modern Approaches to Diagnosis and Treatment. Semin Thromb Hemost. 2020 Apr;46(3):275-288.
[5]	Cooper N, Ghanima W, Hill QA, Nicolson PL, Markovtsov V, Kessler C. Recent advances in understanding spleen tyrosine kinase (SYK) in human biology and disease, with a focus on fostamatinib. Platelets. 2023 Dec;34(1):2131751.
[6]	Provan D, Stasi R, Newland AC, et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood. 2010;115(2):168-186.
[7]	Fattizzo B, Cecchi N, Bortolotti M, Giordano G, Patriarca A, Glenthøj A, Cantoni S, Capecchi M, Chen F, Mingot-Castellano ME, Napolitano M, Frederiksen H, Gonzaléz-Lopez TJ, Barcellini W. Thrombopoietin receptor agonists in adult Evans syndrome: an international multicenter experience. Blood. 2022 Aug 18;140(7):789-792.