Combination of Evans syndrome and COVID-19: a systematic review of reported cases

Evans syndrome is a rare autoimmune disease characterized by simultaneous or sequential primary immune thrombocytopenia and autoimmune hemolytic anemia. Despite the low incidence of Evans syndrome after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, its progression may threaten public health. This review offers an up-to-date summary of the works on the association between coronavirus disease 2019 (COVID-19) and Evans syndrome to explore the pathogenic mechanisms, epidemiological characteristics, clinical presentations, diagnostic markers, and treatment strategies.

We searched PubMed and Web of Science to identify articles that explored the relationship between COVID-19 and Evans syndrome. We collected and organized all reported cases of Evans syndrome following COVID-19 or SARS-CoV-2 vaccination over the past 4 years and also expanded the search to examine other cases of post-infection Evans syndrome.

Thirteen cases were included with an average age of 42 years of whom 12 survived and one died. Two cases were associated with pregnancy and four with vaccination, two involved epileptic seizures, and three had a history of autoimmune disease.

Patients with Evans syndrome and exposure to SARS-CoV-2 have a potential risk of bleeding. This risk should prompt close monitoring of bleeding biomarker dynamics and early initiation of hemostatic treatments, including platelet transfusion, corticosteroids, thrombopoietin receptor agonists, intravenous immunoglobulin and rituximab.