Department of Nephrology, First Faculty of Medicine, General University Hospital, Charles University, Prague, Czechia.
University Hospital, Charles University - Faculty of Medicine, Hradec Králové, Czechia.
Front Immunol. 2022 Sep 28;13:1001366. doi: 10.3389/fimmu.2022.1001366. eCollection 2022.
Atypical hemolytic uremic syndrome (aHUS), also called complement-mediated hemolytic uremic syndrome (CM-HUS), is a rare disease caused by dysregulation in the alternative complement activation pathway. It is a life-threatening condition causing ischemia of a number of organs, and it typically causes acute kidney injury. This disorder may be triggered by various factors including viral or bacterial infections, pregnancy, surgery, and injuries. In about 60% of cases, the genetic origin of the disease can be identified-commonly mutations affecting complementary factor H and MCP protein. Eculizumab, a monoclonal antibody to the C5 component of the complement, represents the current effective treatment.We describe a case of a young woman with a previous history of polyvalent allergies, who developed atypical hemolytic uremic syndrome after vaccination with mRNA vaccine against SARS-CoV-2. The disease manifested by scleral bleeding, acute renal insufficiency, anemia, and thrombocytopenia. The patient was treated with without sufficient effect; remission occurred only after starting treatment with eculizumab. Genetic examination showed that the patient is a carrier of multiple inherited risk factors (a rare pathogenic variant in , MCPggaac haplotype of the gene, and the risk haplotype H3). The patient is currently in hematological remission with persistent mild renal insufficiency, continuing treatment with eculizumab/ravulizumab. By this case report, we meant to point out the need for careful monitoring of people after vaccination, as it may trigger immune-mediated diseases, especially in those with predisposing factors.
非典型溶血尿毒症综合征(aHUS),也称补体介导的溶血尿毒症综合征(CM-HUS),是一种由替代补体激活途径失调引起的罕见疾病。它是一种危及生命的疾病,可导致多个器官缺血,通常会导致急性肾损伤。这种疾病可能由多种因素引发,包括病毒或细菌感染、妊娠、手术和外伤等。约 60%的病例可以确定疾病的遗传起源,常见的突变影响补体因子 H 和 MCP 蛋白。依库珠单抗,一种针对补体 C5 成分的单克隆抗体,是目前有效的治疗方法。我们描述了一例年轻女性,有多种过敏的既往病史,在接种 SARS-CoV-2 mRNA 疫苗后发生非典型溶血尿毒症综合征。疾病表现为巩膜出血、急性肾功能不全、贫血和血小板减少。该患者接受了 治疗,但效果不佳;仅在开始使用依库珠单抗治疗后才缓解。基因检查显示该患者是多种遗传风险因素的携带者(罕见的致病性变异在 基因中,MCPggaac 基因的单倍型,以及风险单倍型 H3)。该患者目前处于血液学缓解期,但持续存在轻度肾功能不全,继续接受依库珠单抗/拉维珠单抗治疗。通过本病例报告,我们旨在指出需要仔细监测接种后的人群,因为疫苗接种可能会引发免疫介导的疾病,尤其是在有易感因素的人群中。
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