Department of Nephrology and Medical Intensive Care, Charité - Universitätsmedizin Berlin, Berlin, Germany.
Department of Nephropathology, Friedrich-Alexander University, Erlangen, Nürnberg, Germany.
BMC Nephrol. 2021 Oct 12;22(1):337. doi: 10.1186/s12882-021-02538-0.
Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening disease characterized by hyperactivation of the immune system that causes hypercytokinemia and potentially multi organ failure. HLH can occur in patients with underlying rheumatic or autoinflammatory disorders. Additionally, HLH can develop in patients during infections or malignancies without a known genetic predisposition.
We herein report a patient, who presented with fever, both acute kidney and liver injury, anemia, thrombocytopenia and HSV stomatitis. HLH was diagnosed based on clinical criteria and qPCR revealed an acute parvovirus B19 infection as potential underlying infectious trigger. Treatment was started with both IVIG and dexamethasone. Subsequently, kidney biopsy demonstrated TMA.
In rare cases both HLH and aHUS can occur simultaneously in a patient as a consequence of viral infections. Insights from this unusual case might help physicians understand this complex symptom constellation.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见且危及生命的疾病，其特征为免疫系统的过度激活，导致细胞因子血症，并可能导致多器官衰竭。HLH 可发生于存在潜在风湿性或自身炎症性疾病的患者中。此外，HLH 也可发生于感染或恶性肿瘤患者中，而无已知的遗传易感性。
我们在此报告一例患者，其表现为发热、急性肾和肝损伤、贫血、血小板减少和单纯疱疹性口炎。根据临床标准诊断为 HLH，qPCR 显示急性细小病毒 B19 感染可能是潜在的感染触发因素。开始采用 IVIG 和地塞米松进行治疗。随后，肾活检显示 TMA。
在罕见情况下，病毒感染可导致 HLH 和 aHUS 同时在患者中发生。从这个不寻常的病例中获得的认识可能有助于医生理解这种复杂的症状组合。
