产气荚膜梭菌菌血症是一种罕见但迅速致命的疾病，特别是对于表现出大量血管内溶血 (MIH)、气性坏疽和感染性休克的患者。在此，我们介绍一例产气荚膜梭菌败血症的尸检病例，表现出 MIH、气性坏疽和细胞因子风暴。患者为84岁女性，有先天性胆道扩张症胆道重建手术史。她患上了 MIH、炎症介质水平升高、血小板减少症和凝血病。她在演讲后 1 小时内陷入休克，并在几个小时内死亡。计算机断层扫描显示，快速进展与肝脓肿转变为充满气体的脓肿有关，这表明产气细菌的快速生长。根据血液和胆汁中存在产气荚膜梭菌感染，该患者最终被诊断为 MIH 和气性坏疽。尸检时，几乎所有器官都观察到源自胆管的气性坏疽。针对产气荚膜梭菌毒素的聚合酶链反应鉴定出分离的细菌为 A 型产气荚膜梭菌，表达 α-毒素 (CPA)、产气荚膜溶血素 O (PFO) 和胶原酶 (ColA)。血清中观察到白细胞介素 6 和肿瘤坏死因子-α 表达水平升高，这种促炎反应部分由 Toll 样受体 2 介导。这项研究阐明了临床分离的产气荚膜梭菌毒素谱与宿主细胞因子之间的关联患者的反应。© 2024 作者。

Clostridium perfringens bacteremia is a rare but rapidly fatal condition, especially in patients exhibiting massive intravascular hemolysis (MIH), gas gangrene, and septic shock. Herein, we present an autopsy case of C. perfringens septicemia exhibiting MIH, gas gangrene, and cytokine storm. The patient was an 84-year-old female with a history of biliary reconstruction surgery for congenital biliary dilatation. She developed MIH, elevated inflammatory mediator levels, thrombocytopenia, and coagulopathy. She went into shock within 1 h of the presentation and died within a few hours. Rapid progression was associated with the transformation of liver abscesses into gas-filled abscesses on computed tomography scan, suggesting the rapid outgrowth of gas-producing bacteria. The patient was finally diagnosed with MIH and gas gangrene due to C. perfringens infection based on the presence of this bacterium in the blood and bile. On autopsy, gas gangrene was observed in almost all organs, originating from the bile duct. Polymerase chain reactions targeting C. perfringens toxins identified the isolated bacterium as C. perfringens type A expressing α-toxin (CPA), perfringolysin O (PFO), and collagenase (ColA). Elevated interleukin 6 and tumor necrosis factor-α expression levels were observed in the serum, and such proinflammatory responses were partially mediated by Toll-like receptor 2. This study elucidated the association between the toxin profiles of clinically isolated C. perfringens and the host cytokine responses in the patient.© 2024 The Authors.