Yacoub Andrew, Atalla Michael, Hasnaoui Anis, Ramdass Prakash V A K
St. George's University School of Medicine, True Blue Campus, St. George, Grenada.
Faculty of Medicine of Tunis, Tunis El Manar University, Tunis, Tunisia.
J Hematol. 2025 Jun;14(3):109-123. doi: 10.14740/jh2058. Epub 2025 May 13.
Evans syndrome (ES) is an autoimmune disorder of unknown etiology characterized by autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). In this systematic review, we analyzed the reported cases of ES secondary to coronavirus disease 2019 (COVID-19) infection or COVID-19 vaccination. We examined their clinical presentation, temporality between events, diagnostics tests, and treatment regimens. Our search in four databases from December 2019 to September 2023 yielded 16 case reports that met eligibility criteria for inclusion. COVID-19 and ES symptoms were defined to assess the timeline between infection/vaccination and ES onset. Finally, treatment efficacy was categorized as complete, partial, or no response based on standard hematological criteria. Eleven cases of ES were associated with COVID-19 infection, and five cases of ES were associated with COVID-19 vaccination. All 16 cases presented with anemia, thrombocytopenia, and a positive Coombs test. Four of the five patients from the vaccination subset were found to have an additional autoimmune disease as a comorbidity on presentation. For cases of ES secondary to COVID-19 infection, six patients had concomitant symptoms of COVID-19 and ES on presentation, and four patients had ES symptoms occurring from 5 days to 3 weeks following COVID-19 infection. The remaining case presented a patient with a 3-week history of ES symptoms before a positive COVID-19 test and further ES workup on admission. For the five cases of ES post-COVID-19 vaccination, all five patients presented with ES with a mean presentation time of 9 days following vaccination. Regarding treatment, intravenous immunoglobulin (IVIG) emerged as the primary regimen, administered in 13 out of the 16 cases. Among the infection-related cases, the most frequent treatment outcome was a partial response in both AIHA and ITP, observed in five of the 11 patients. In the vaccination-related cases, a partial response for AIHA and a complete response for ITP were noted in three of the five patients. Overall, while the evidence points to a temporal association especially between COVID-19 vaccination and the onset of ES, larger studies are necessary to strengthen these findings. In terms of management, early initiation of corticosteroids and IVIG appears effective as first-line therapies; however, standardized treatment protocols are needed to help reduce complications associated with COVID-19-related ES.
伊文氏综合征（ES）是一种病因不明的自身免疫性疾病，其特征为自身免疫性溶血性贫血（AIHA）和免疫性血小板减少症（ITP）。在本系统评价中，我们分析了报告的继发于2019冠状病毒病（COVID-19）感染或COVID-19疫苗接种的ES病例。我们研究了它们的临床表现、事件之间的时间关系、诊断测试和治疗方案。我们在2019年12月至2023年9月期间对四个数据库进行检索，得到了16篇符合纳入标准的病例报告。定义了COVID-19和ES症状，以评估感染/接种疫苗与ES发病之间的时间线。最后，根据标准血液学标准，将治疗效果分为完全缓解、部分缓解或无反应。11例ES与COVID-19感染相关，5例ES与COVID-19疫苗接种相关。所有16例均出现贫血、血小板减少和抗人球蛋白试验阳性。疫苗接种亚组的5例患者中有4例在就诊时发现有其他自身免疫性疾病作为合并症。对于继发于COVID-19感染的ES病例，6例患者在就诊时同时出现COVID-19和ES症状，4例患者在COVID-19感染后5天至3周出现ES症状。其余1例患者在COVID-19检测呈阳性及入院进一步进行ES检查之前，已有3周的ES症状病史。对于5例COVID-19疫苗接种后发生的ES病例，所有5例患者均出现ES，平均发病时间为接种疫苗后9天。关于治疗，静脉注射免疫球蛋白（IVIG）成为主要治疗方案，16例中有13例使用。在与感染相关的病例中，最常见的治疗结果是11例患者中有5例在AIHA和ITP中出现部分缓解。在与疫苗接种相关的病例中，5例患者中有3例在AIHA中出现部分缓解，在ITP中出现完全缓解。总体而言，虽然证据表明特别是在COVID-19疫苗接种与ES发病之间存在时间关联，但需要更大规模的研究来强化这些发现。在管理方面，早期使用皮质类固醇和IVIG作为一线治疗似乎有效；然而，需要标准化的治疗方案来帮助减少与COVID-19相关的ES的并发症。
