A Case of Evans Syndrome and Unstable Angina

Saad Bin Jamil, Iqra Patoli, Mehrunissa Kazim, Syed Hassan Abbas, Zain Ali

Abstract


Evans syndrome (ES) is characterized by autoimmune hemolytic anemia (AIHA) and immune-mediated thrombocytopenia. It is more common in the pediatric population than in adults. ES has been reported to be associated with thrombotic events and rarely can lead to acute coronary syndrome (ACS). There have been only a few reported cases of ACS secondary to ES. We present an interesting case of ES with unstable angina (UA) which had a limited response to oral and intravenous (IV) steroids requiring rituximab. A 64-year-old male with past medical history significant for hypertension, hyperlipidemia, diabetes mellitus and coronary artery disease, presented to the emergency room complaining of a 2-week history of chest pain, shortness of breath and hematuria. Physical examination indicated splenomegaly but was otherwise unremarkable with no petechiae or rash. Labs showed hemoglobin of 9.6 g/dL, platelet count 58 103/L, troponin < 0.03 ng/mL, lactic acid 2.5 mmol/L and with parameters indicative of hemolysis, evidenced by elevated lactate dehydrogenase, low haptoglobin and elevated bilirubin levels. Electrocardiography (EKG) demonstrated ST depression in leads I, aVL, V5 - V6 and T wave inversions in lead III and aVL, which were new compared to previous EKG. Peripheral blood smear indicated spherocytes. Direct antiglobulin test was positive for immunoglobulin G (IgG). Patient was admitted for ES and initially treated with oral prednisone 80 mg daily. He was also diagnosed with UA thought to be possibly secondary to ES. He then underwent cardiac stress test which showed mild reversible inferior apical ischemia. Cardiac catheterization revealed 95% stenosis of proximal left circumflex artery requiring single drug eluding stent placement and dual antiplatelet therapy. Patient continued to have anemia despite blood transfusions, although platelet count improved. Prednisone was transitioned to high-dose IV dexamethasone, and patient was also started on rituximab which resulted in stabilization of anemia. The presentation of ES with ACS is a rare occurrence. ACS can be challenging to manage as stent placement may be required followed by dual antiplatelet therapy. Treatment of ES involves steroids followed by rituximab, IV immunoglobulin (IVIG) or splenectomy for non-responsive cases. Early intervention and management can prevent mortality and morbidity.




J Med Cases. 2021;12(10):415-418
doi: https://doi.org/10.14740/jmc3777

Keywords


Evans syndrome; Unstable angina; Autoimmune hemolysis; Thrombosis; Thrombocytopenia; Intravenous immunoglobulin; Rituximab

Full Text: HTML PDF
 

Browse  Journals  

 

Journal of Clinical Medicine Research

Journal of Endocrinology and Metabolism

Journal of Clinical Gynecology and Obstetrics

 

World Journal of Oncology

Gastroenterology Research

Journal of Hematology

 

Journal of Medical Cases

Journal of Current Surgery

Clinical Infection and Immunity

 

Cardiology Research

World Journal of Nephrology and Urology

Cellular and Molecular Medicine Research

 

Journal of Neurology Research

International Journal of Clinical Pediatrics

 

 
       
 

Journal of Medical Cases, monthly, ISSN 1923-4155 (print), 1923-4163 (online), published by Elmer Press Inc.                     
The content of this site is intended for health care professionals.
This is an open-access journal distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License, which permits unrestricted
non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Creative Commons Attribution license (Attribution-NonCommercial 4.0 International CC-BY-NC 4.0)


This journal follows the International Committee of Medical Journal Editors (ICMJE) recommendations for manuscripts submitted to biomedical journals,
the Committee on Publication Ethics (COPE) guidelines, and the Principles of Transparency and Best Practice in Scholarly Publishing.

website: www.journalmc.org   editorial contact: editor@journalmc.org    elmer.editorial@hotmail.com
Address: 9225 Leslie Street, Suite 201, Richmond Hill, Ontario, L4B 3H6, Canada

© Elmer Press Inc. All Rights Reserved.


Disclaimer: The views and opinions expressed in the published articles are those of the authors and do not necessarily reflect the views or opinions of the editors and Elmer Press Inc. This website is provided for medical research and informational purposes only and does not constitute any medical advice or professional services. The information provided in this journal should not be used for diagnosis and treatment, those seeking medical advice should always consult with a licensed physician.