Relapse of Evans syndrome following BNT162b2 (Pfizer-BioNTech) COVID-19 vaccine: case report and literature review

Introduction: Coronavirus disease 2019 (COVID-19) vaccines are considered to be safe. Only few cases of vaccine-induced immune thrombocytopenia or immune hemolysis have been reported so far. Evans syndrome (ES) is a very rare syndrome characterized mainly by warm autoimmune hemolytic anemia (wAIHA)...

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Main Authors: Mirjana Cvetković, Nikola Pantić, Marijana Virijević, Zlatko Pravdić, Nikica Sabljić, Mirjana Mitrović, Nada Suvajdžić-Vuković
Format: Article
Language:English
Published: The Journal of Infection in Developing Countries 2023-06-01
Series:Journal of Infection in Developing Countries
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Online Access:https://jidc.org/index.php/journal/article/view/17719
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author Mirjana Cvetković
Nikola Pantić
Marijana Virijević
Zlatko Pravdić
Nikica Sabljić
Mirjana Mitrović
Nada Suvajdžić-Vuković
author_facet Mirjana Cvetković
Nikola Pantić
Marijana Virijević
Zlatko Pravdić
Nikica Sabljić
Mirjana Mitrović
Nada Suvajdžić-Vuković
author_sort Mirjana Cvetković
collection DOAJ
description Introduction: Coronavirus disease 2019 (COVID-19) vaccines are considered to be safe. Only few cases of vaccine-induced immune thrombocytopenia or immune hemolysis have been reported so far. Evans syndrome (ES) is a very rare syndrome characterized mainly by warm autoimmune hemolytic anemia (wAIHA) and immune thrombocytopenia (ITP). Case presentation: We present a case of a 47‐year‐old male with a history of wAIHA, diagnosed in 1995 and successfully treated with glucocorticoids, with sustained remission. ITP was diagnosed in May 2016. Due to refractoriness to glucocorticoids, intravenous immunoglobulins (IVIGs), azathioprine and vinblastine, he was splenectomised in April 2017, resulting in complete remission. In May 2021, eight days after the second dose of BNT162b2 (Pfizer-BioNTech) COVID-19 vaccine, he experienced mucocutaneous bleeding. Blood tests showed platelet count (PC) of 8×109/L, while his hemoglobin (Hb) was normal (153 g/L). He was treated with prednisone and azathioprine, without response. On day 28 after vaccine administration, weakness, jaundice and dark brown urine occurred. His laboratory tests: PC 27×109/L, Hb 45 g/L, reticulocytes 10.4%, total bilirubin 106.6 μmol/L, direct bilirubin 19.8 μmol/L, lactate dehydrogenase 633 U/L, haptoglobin ˂0.08 g/L, and positive Coombs test were consistent with ES relapse. After treatment with glucocorticoids, azathioprine and IVIGs, his blood count finally improved (PC 490×109/L, Hb 109 g/L) and remained stable on day 40 of hospitalization. Conclusions: Although it is unclear whether the relationship between COVID-19 vaccination and relapse of ES in our patient is coincidental or causal, it highlights the need for monitoring of serious outcomes following vaccination.
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publisher The Journal of Infection in Developing Countries
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spelling doaj-art-2bcb488e4c0841a9aca508911e8cec1c2025-08-20T02:16:17ZengThe Journal of Infection in Developing CountriesJournal of Infection in Developing Countries1972-26802023-06-01170610.3855/jidc.17719Relapse of Evans syndrome following BNT162b2 (Pfizer-BioNTech) COVID-19 vaccine: case report and literature reviewMirjana Cvetković0Nikola Pantić1Marijana Virijević2Zlatko Pravdić3Nikica Sabljić4Mirjana Mitrović5Nada Suvajdžić-Vuković6Clinic of Hematology, University Clinical Center of Serbia, Belgrade, SerbiaClinic of Hematology, University Clinical Center of Serbia, Belgrade, SerbiaClinic of Hematology, University Clinical Center of Serbia, Belgrade, SerbiaClinic of Hematology, University Clinical Center of Serbia, Belgrade, SerbiaClinic of Hematology, University Clinical Center of Serbia, Belgrade, SerbiaClinic of Hematology, University Clinical Center of Serbia, Belgrade, SerbiaClinic of Hematology, University Clinical Center of Serbia, Belgrade, Serbia Introduction: Coronavirus disease 2019 (COVID-19) vaccines are considered to be safe. Only few cases of vaccine-induced immune thrombocytopenia or immune hemolysis have been reported so far. Evans syndrome (ES) is a very rare syndrome characterized mainly by warm autoimmune hemolytic anemia (wAIHA) and immune thrombocytopenia (ITP). Case presentation: We present a case of a 47‐year‐old male with a history of wAIHA, diagnosed in 1995 and successfully treated with glucocorticoids, with sustained remission. ITP was diagnosed in May 2016. Due to refractoriness to glucocorticoids, intravenous immunoglobulins (IVIGs), azathioprine and vinblastine, he was splenectomised in April 2017, resulting in complete remission. In May 2021, eight days after the second dose of BNT162b2 (Pfizer-BioNTech) COVID-19 vaccine, he experienced mucocutaneous bleeding. Blood tests showed platelet count (PC) of 8×109/L, while his hemoglobin (Hb) was normal (153 g/L). He was treated with prednisone and azathioprine, without response. On day 28 after vaccine administration, weakness, jaundice and dark brown urine occurred. His laboratory tests: PC 27×109/L, Hb 45 g/L, reticulocytes 10.4%, total bilirubin 106.6 μmol/L, direct bilirubin 19.8 μmol/L, lactate dehydrogenase 633 U/L, haptoglobin ˂0.08 g/L, and positive Coombs test were consistent with ES relapse. After treatment with glucocorticoids, azathioprine and IVIGs, his blood count finally improved (PC 490×109/L, Hb 109 g/L) and remained stable on day 40 of hospitalization. Conclusions: Although it is unclear whether the relationship between COVID-19 vaccination and relapse of ES in our patient is coincidental or causal, it highlights the need for monitoring of serious outcomes following vaccination. https://jidc.org/index.php/journal/article/view/17719Evans syndromerelapseCOVID-19 vaccination
spellingShingle Mirjana Cvetković
Nikola Pantić
Marijana Virijević
Zlatko Pravdić
Nikica Sabljić
Mirjana Mitrović
Nada Suvajdžić-Vuković
Relapse of Evans syndrome following BNT162b2 (Pfizer-BioNTech) COVID-19 vaccine: case report and literature review
Journal of Infection in Developing Countries
Evans syndrome
relapse
COVID-19
vaccination
title Relapse of Evans syndrome following BNT162b2 (Pfizer-BioNTech) COVID-19 vaccine: case report and literature review
title_full Relapse of Evans syndrome following BNT162b2 (Pfizer-BioNTech) COVID-19 vaccine: case report and literature review
title_fullStr Relapse of Evans syndrome following BNT162b2 (Pfizer-BioNTech) COVID-19 vaccine: case report and literature review
title_full_unstemmed Relapse of Evans syndrome following BNT162b2 (Pfizer-BioNTech) COVID-19 vaccine: case report and literature review
title_short Relapse of Evans syndrome following BNT162b2 (Pfizer-BioNTech) COVID-19 vaccine: case report and literature review
title_sort relapse of evans syndrome following bnt162b2 pfizer biontech covid 19 vaccine case report and literature review
topic Evans syndrome
relapse
COVID-19
vaccination
url https://jidc.org/index.php/journal/article/view/17719
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