New-onset Evans syndrome associated with systemic lupus erythematosus after BNT162b2 mRNA COVID-19 vaccination

Hidaka, Daisuke; Ogasawara, Reiki; Sugimura, Shunsuke; Fujii, Fumiaki; Kojima, Keisuke; Nagai, Jun; Ebata, Ko; Okada, Kohei; Kobayashi, Naoki; Ogasawara, M.; Imamura, Masahiro; Ota, Shuichi

doi:10.1007/s12185-021-03243-2

Cited by 30 publications

(28 citation statements)

References 24 publications

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“…On the other hand, in a cohort of 56 patients with a history of AIHA, four elderly patients experienced a clinically significant hemoglobin reduction requiring treatment adjustment after receiving mRNA-based vaccines BNT162b2 or mRNA-1273. The same report described seven patients with autoimmune neutropenia who did not have exacerbations after receiving mRNA-based vaccines [110].…”

Section: Covid-19 Covid-19 Vaccination and Aiha Or Evans Syndromementioning

confidence: 94%

“…On the other hand, the number of statements documenting the association between COVID-19 vaccines and Evans syndrome is remarkably low (Table 3). To the best of our knowledge, only one of these cases is described in the literature, corresponding to a female patient who also presented with systemic lupus erythematosus (SLE) after being administered the BNT162b2 vaccine [110].…”

Section: Covid-19 Covid-19 Vaccination and Aiha Or Evans Syndromementioning

confidence: 99%

“…A young woman whose case was particularly severe was transfused with 13 units of packed RBCs, and administered corticosteroids, rituximab, mycophenolate mofetil, and immune globulin [108]. Finally, the patient who presented with Evans syndrome and SLE after having received the BNT162b2 vaccine was administered prednisolone to rapidly restore hemoglobin levels and platelet count [110].…”

Section: Management Of Aiha and Evans Syndromementioning

confidence: 99%

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COVID-19 Vaccines and Autoimmune Hematologic Disorders

et al. 2022

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Worldwide vaccination against SARS-CoV-2 has allowed the detection of hematologic autoimmune complications. Adverse events (AEs) of this nature had been previously observed in association with other vaccines. The underlying mechanisms are not totally understood, although mimicry between viral and self-antigens plays a relevant role. It is important to remark that, although the incidence of these AEs is extremely low, their evolution may lead to life-threatening scenarios if treatment is not readily initiated. Hematologic autoimmune AEs have been associated with both mRNA and adenoviral vector-based SARS-CoV-2 vaccines. The main reported entities are secondary immune thrombocytopenia, immune thrombotic thrombocytopenic purpura, autoimmune hemolytic anemia, Evans syndrome, and a newly described disorder, so-called vaccine-induced immune thrombotic thrombocytopenia (VITT). The hallmark of VITT is the presence of anti-platelet factor 4 autoantibodies able to trigger platelet activation. Patients with VITT present with thrombocytopenia and may develop thrombosis in unusual locations such as cerebral beds. The management of hematologic autoimmune AEs does not differ significantly from that of these disorders in a non-vaccine context, thus addressing autoantibody production and bleeding/thromboembolic risk. This means that clinicians must be aware of their distinctive signs in order to diagnose them and initiate treatment as soon as possible.

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Section: Covid-19 Covid-19 Vaccination and Aiha Or Evans Syndromementioning

confidence: 94%

Section: Covid-19 Covid-19 Vaccination and Aiha Or Evans Syndromementioning

confidence: 99%

Section: Management Of Aiha and Evans Syndromementioning

confidence: 99%

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COVID-19 Vaccines and Autoimmune Hematologic Disorders

et al. 2022

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“…Anyway, to our knowledge, this is not the first evidence of the association of ES with SARS-CoV-2 vaccination. Hidaka et al have reported a new-onset ES with systemic lupus erythematosus after a second dose of BNT162b2 mRNA COVID-19 vaccination, quickly resolved with prednisolone 1 mg/kg/day [15]. Specifically, our case describes an isolated secondary ES after a single dose of SARS-CoV-2 vaccine, demonstrating a rapid resolution of AIHA although with the persistence of ITP, which generally tends to be more sensitive to immunosuppressive drugs than AIHA.…”

Section: Discussionmentioning

confidence: 58%

Evans Syndrome Presenting as an Atypical Complication of SARS-CoV-2 Vaccination

Felice¹,

Farina²,

Bianco³

et al. 2022

Cureus

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The coronavirus disease 2019 (COVID-19) pandemic, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has drastically affected our daily lives, causing millions of deaths worldwide. The early and late complications of this infection are being increasingly revealed on a regular basis; however, an important brake on the spread and especially the lethality of the disease has been guaranteed by the introduction of mRNA-based and viral vector-based COVID-19 Vaccines. Also, an increasing number of adverse effects of the vaccination have been reported to specific pharmacovigilance boards, most of them totally non-serious events that are resolved within one to three days after the administration of the vaccine. In this report, we present a case of Evans syndrome (ES) secondary to SARS-CoV-2 vaccination in an 85-year-old male patient. To the best of our knowledge, this is the first case of ES caused by the COVID-19 vaccination to be reported in the literature.

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“…Complications such as hematuria, extensive petechial hemorrhage, subarachnoid hemorrhage [ 178 ], immune thrombocytopenia [ 179 , 180 , 181 , 182 , 183 ], Evans syndrome [ 184 ], acquired hemophilia [ 185 ], and factor XIII inhibitor [ 186 ] have been reported with the use of adenovirus vector and mRNA vaccines. In particular, when platelet transfusion is performed for hemorrhagic disease, examination to rule out VITT should always be performed, because with the presence of VITT in the background, the underlying condition may be aggravated after platelet transfusion.…”

Section: Sars-cov-2 Vaccination-associated Coagulopathymentioning

confidence: 99%

Coagulopathy and Fibrinolytic Pathophysiology in COVID-19 and SARS-CoV-2 Vaccination

Yamada

Asakura

2022

IJMS

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Coronavirus Disease 2019 (COVID-19) is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and is frequently complicated by thrombosis. In some cases of severe COVID-19, fibrinolysis may be markedly enhanced within a few days, resulting in fatal bleeding. In the treatment of COVID-19, attention should be paid to both coagulation activation and fibrinolytic activation. Various thromboses are known to occur after vaccination with SARS-CoV-2 vaccines. Vaccine-induced immune thrombotic thrombocytopenia (VITT) can occur after adenovirus-vectored vaccination, and is characterized by the detection of anti-platelet factor 4 antibodies by enzyme-linked immunosorbent assay and thrombosis in unusual locations such as cerebral venous sinuses and visceral veins. Treatment comprises high-dose immunoglobulin, argatroban, and fondaparinux. Some VITT cases show marked decreases in fibrinogen and platelets and marked increases in D-dimer, suggesting the presence of enhanced-fibrinolytic-type disseminated intravascular coagulation with a high risk of bleeding. In the treatment of VITT, evaluation of both coagulation activation and fibrinolytic activation is important, adjusting treatments accordingly to improve outcomes.

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New-onset Evans syndrome associated with systemic lupus erythematosus after BNT162b2 mRNA COVID-19 vaccination

Cited by 30 publications

References 24 publications

COVID-19 Vaccines and Autoimmune Hematologic Disorders

COVID-19 Vaccines and Autoimmune Hematologic Disorders

Evans Syndrome Presenting as an Atypical Complication of SARS-CoV-2 Vaccination

Coagulopathy and Fibrinolytic Pathophysiology in COVID-19 and SARS-CoV-2 Vaccination

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