van Herpt Thijs T W, Timmermans Sjoerd A M E G, van Mook Walther N K A, van Bussel Bas C T, van der Horst Iwan C C, Maessen Jos G, Natour Ehsan, van Paassen Pieter, Heuts Samuel
Department of Intensive Care Medicine, Maastricht University Medical Center+, 6229 HX Maastricht, The Netherlands.
Department of Nephrology and Clinical Immunology, Maastricht University Medical Center+, 6229 HX Maastricht, The Netherlands.
J Clin Med. 2022 Apr 29;11(9):2501. doi: 10.3390/jcm11092501.
Postsurgical thrombotic microangiopathy (TMA) is a complication associated with significant morbidity and mortality. Still, the pathophysiological underlying mechanism of postsurgical TMA, a diagnosis often overlooked in postoperative patients with acute kidney injury and thrombocytopenia, is largely unknown. Here, we report the case of a 56-year-old male that developed anuric acute kidney injury, Coombs-negative hemolysis, and thrombocytopenia after surgical aortic arch replacement. Massive ex vivo complement activation on the endothelium, a rare complement gene variant in , at-risk haplotype ggaac, and excellent response to therapeutic complement inhibition, points to the pivotal role of complement in the pathophysiology of disease. Moreover, the importance of a multidisciplinary team approach in (postsurgical) thrombocytopenia is emphasized.
术后血栓性微血管病（TMA）是一种与显著发病率和死亡率相关的并发症。然而，术后TMA的病理生理潜在机制在很大程度上尚不清楚，这一诊断在急性肾损伤和血小板减少的术后患者中常常被忽视。在此，我们报告一例56岁男性患者，在接受主动脉弓置换手术后出现无尿性急性肾损伤、库姆斯试验阴性溶血和血小板减少。内皮细胞上大量的体外补体激活、一种罕见的补体基因变异、高危单倍型ggaac以及对治疗性补体抑制的良好反应，表明补体在疾病病理生理学中起关键作用。此外，强调了多学科团队方法在（术后）血小板减少症中的重要性。
