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Postsurgical Thrombotic Microangiopathy and Deregulated Complement

Thijs T W van Herpt1, Sjoerd A M E G Timmermans2, Walther N K A van Mook1,3

  • 1Department of Intensive Care Medicine, Maastricht University Medical Center+, 6229 HX Maastricht, The Netherlands.

Journal of Clinical Medicine
|May 14, 2022
PubMed

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 C2, at-risk haplotype MCPggaac, 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.

Keywords:
aortic surgerycomplement activationcomplement inhibitiongenetic variancehemolytic uremic syndromemultidisciplinary approachthrombocytopeniathrombotic micro-angiopathy

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