Brink Hannah M, Wallworth Taylor M, Penney Scott W, Padial Javier A, Gale Hannah L
Pediatrics, Brooke Army Medical Center, San Antonio, USA.
Emergency Medicine, David Grant United States Air Force (USAF) Medical Center, Fairfield, USA.
Cureus. 2025 Jul 7;17(7):e87437. doi: 10.7759/cureus.87437. eCollection 2025 Jul.
A previously healthy seven-year-old boy presented with clinical and laboratory findings consistent with atypical hemolytic uremic syndrome (aHUS) given thrombocytopenia, microangiopathic hemolytic anemia (MAHA), and acute kidney injury in the setting of influenza A. Notably, he also met diagnostic criteria for disseminated intravascular coagulation (DIC) at the time of presentation with clinical findings including prolonged prothrombin time (PT), markedly elevated D-dimer, and low fibrinogen. While aHUS and DIC share overlapping clinical features, they are traditionally regarded as distinct entities, with aHUS driven by complement dysregulation and DIC by the widespread activation of the coagulation cascade resulting in microvascular thrombosis, consumptive coagulopathy, and secondary fibrinolysis. The patient was treated with both supportive care and eculizumab, a terminal complement inhibitor, leading to rapid and sustained clinical and laboratory improvement without recurrence. This case highlights the diagnostic and therapeutic complexity of concurrent aHUS and DIC, lends clinical support to emerging hypotheses that uncontrolled complement activation may contribute to DIC, and underscores the value of early recognition and complement-directed therapy in atypical hemolytic uremic syndrome (HUS).
一名此前健康的七岁男孩出现了与非典型溶血尿毒综合征（aHUS）相符的临床和实验室检查结果，表现为血小板减少、微血管病性溶血性贫血（MAHA）以及甲型流感背景下的急性肾损伤。值得注意的是，他在就诊时还符合弥散性血管内凝血（DIC）的诊断标准，临床表现包括凝血酶原时间（PT）延长、D - 二聚体显著升高以及纤维蛋白原降低。虽然aHUS和DIC有一些重叠的临床特征，但传统上它们被视为不同的病症，aHUS由补体失调驱动，而DIC是由凝血级联反应的广泛激活导致微血管血栓形成、消耗性凝血病和继发性纤维蛋白溶解。该患者接受了支持治疗和终末补体抑制剂依库珠单抗治疗，临床和实验室检查结果迅速且持续改善，未复发。本病例突出了并发aHUS和DIC的诊断和治疗复杂性，为未控制的补体激活可能导致DIC这一新兴假说提供了临床支持，并强调了在非典型溶血尿毒综合征（HUS）中早期识别和补体导向治疗的价值。
