Evans Syndrome Probably Secondary to BNT162b2 SARS-CoV-2 Vaccine: Case Report

Authors

  • Inês SF da Silva Serviço de Medicina Interna – Área B, Unidade Local de Saúde Santa Maria, Lisboa, Portugal https://orcid.org/0000-0001-7371-7035
  • Ryan Costa Silva Serviço de Medicina Interna – Área B, Unidade Local de Saúde Santa Maria, Lisboa, Portugal. Clínica Universitária de Medicina Interna, Unidade Local de Saúde Santa Maria, Lisboa, Portugal
  • Inês Sopa Serviço de Medicina Interna – Área B, Unidade Local de Saúde Santa Maria, Lisboa, Portugal
  • Catarina Gregório Serviço de Cardiologia, Departamento de Coração e Vasos, Unidade Local de Saúde Santa Maria, Lisboa, Portugal. Cardiovascular Center of the University of Lisbon (CCUL@RISE), Lisboa, Portugal
  • Lígia Peixoto Serviço de Medicina Interna – Área B, Unidade Local de Saúde Santa Maria, Lisboa, Portugal. Clínica Universitária de Medicina Interna, Unidade Local de Saúde Santa Maria, Lisboa, Portugal

DOI:

https://doi.org/10.60591/crspmi.170

Keywords:

Anemia, Hemolytic, Autoimmune/chemically induced, BNT162 Vaccine, COVID-19 Vaccines

Abstract

Evans syndrome is a rare condition. There are few case
reports of Evans syndrome following SARS-CoV-2 mRNA
vaccine, none in Portugal.

An eighteen-year-old male patient was admitted with
abdominal pain, jaundice, dark urine, and fatigue. The evaluation performed was consistent with warm autoimmune hemolytic anemia, but he later developed autoimmune thrombocytopenia. His medical history was notable for IgA vasculitis at the age of 20 months and SARS--CoV-2 mRNA vaccination nine days before admission. Viral infections, autoimmune/lymphoproliferative diseases, immunodeficiencies, medications and thrombophilia were excluded. Although he did not initially respond to corticosteroids or immunoglobulin, he responded favorably to rituximab and cyclophosphamide.

Given the temporal association and the exclusion of other
causes, we can assume Evans syndrome probably secondary to SARS-CoV-2 mRNA vaccine. It is the first case in Portugal and describes a rare idiosyncrasy. It should not discourage vaccination at the population level.

Downloads

Download data is not yet available.

References

Fattizzo B, Michel M, Giannotta JA, Hansen D, Arguello M, Sutto E, et al. Evans syndrome in adults: an observational multicenter study. Blood Adv. 2021;5:5468-78. doi: 10.1182/bloodadvances.2021005610

Mingot-Castellano ME, Butta N, Canaro M, Del Castillo MS, Sánchez-González B, Jiménez-Bárcenas R, et al. COVID-19 Vaccines and

Autoimmune Hematologic Disorders. Vaccines. 2022;10:961. doi: 10.3390/VACCINES10060961

Jaime-Pérez JC, Elva P, Aguilar-Calderón, Salazar-Cavazos L, Gómez-Almaguer D. Evans syndrome: Clinical perspectives, biological insights and treatment modalities. J Blood Med. 2018;9:171-84. doi: 10.2147/JBM.S176144

Fattizzo B. Evans syndrome in the SARS-CoV-2 era: “springing up like mushrooms.” Blood Transfusion. 2022;20:89-93. doi: 10.2450/2021.0224-21

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;94:1081-90. doi: 10.1002/ajh.25574

Hidaka D, Ogasawara R, Sugimura S, Fujii F, Kojima K, Nagai J, et al. New-onset Evans syndrome associated with systemic lupus erythematosus after BNT162b2 mRNA COVID-19 vaccination. Int J Hematol. 2022;115:424-7. doi: 10.1007/s12185-021-03243-2

De Felice M, Farina G, Bianco R, Monaco G, Iaccarino S. Evans Syndrome Presenting as an Atypical Complication of SARS-CoV-2 Vaccination. Cureus. 2022; 14:e26602. doi: 10.7759/cureus.26602.

Michel M. Adult Evans’ Syndrome. Hematol Oncol Clin North Am. 2022;36:381-92. doi: 10.1016/J.HOC.2021.12.004

Audia S, Grienay N, Mounier M, Michel M, Bonnotte B. Evans’ syndrome: From diagnosis to treatment. J Clin Med. 2020;9:1-22. doi:

3390/jcm9123851

Jäger U, Barcellini W, Broome CM, Gertz M, Hill A, Hill Q, et al. Diagnosis and treatment of autoimmune hemolytic anemia in adults: Recommendations from the First International Consensus Meeting. Blood Rev. 2020;41. doi: 10.1016/j.blre.2019.100648

Published

24-06-2024

How to Cite

Silva, I. S. da, Ryan Costa Silva, Sopa, I., Gregório, C., & Peixoto, L. (2024). Evans Syndrome Probably Secondary to BNT162b2 SARS-CoV-2 Vaccine: Case Report. SPMI Case Reports, 2(Edição Especial), 8–14. https://doi.org/10.60591/crspmi.170