{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,11]],"date-time":"2026-07-11T16:19:11Z","timestamp":1783786751371,"version":"3.55.0"},"reference-count":23,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2022,4,29]],"date-time":"2022-04-29T00:00:00Z","timestamp":1651190400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>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.<\/jats:p>","DOI":"10.3390\/jcm11092501","type":"journal-article","created":{"date-parts":[[2022,4,30]],"date-time":"2022-04-30T05:44:07Z","timestamp":1651297447000},"page":"2501","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Postsurgical Thrombotic Microangiopathy and Deregulated Complement"],"prefix":"10.3390","volume":"11","author":[{"given":"Thijs T. W.","family":"van Herpt","sequence":"first","affiliation":[{"name":"Department of Intensive Care Medicine, Maastricht University Medical Center+, 6229 HX Maastricht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5925-2753","authenticated-orcid":false,"given":"Sjoerd A. M. E. G.","family":"Timmermans","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Clinical Immunology, Maastricht University Medical Center+, 6229 HX Maastricht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Walther N. K. A.","family":"van Mook","sequence":"additional","affiliation":[{"name":"Department of Intensive Care Medicine, Maastricht University Medical Center+, 6229 HX Maastricht, The Netherlands"},{"name":"Academy for Postgraduate Medical Training, Maastricht University Medical Center+, 6229 HX Maastricht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1621-7848","authenticated-orcid":false,"given":"Bas C. T.","family":"van Bussel","sequence":"additional","affiliation":[{"name":"Department of Intensive Care Medicine, Maastricht University Medical Center+, 6229 HX Maastricht, The Netherlands"},{"name":"Care and Public Health Research Institute (CAPHRI), Maastricht University, 6229 HX Maastricht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iwan C. C.","family":"van der Horst","sequence":"additional","affiliation":[{"name":"Department of Intensive Care Medicine, Maastricht University Medical Center+, 6229 HX Maastricht, The Netherlands"},{"name":"Cardiovascular Research Institute Maastricht (CARIM), Maastricht University, 6229 HX Maastricht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jos G.","family":"Maessen","sequence":"additional","affiliation":[{"name":"Cardiovascular Research Institute Maastricht (CARIM), Maastricht University, 6229 HX Maastricht, The Netherlands"},{"name":"Department of Cardiothoracic Surgery, Maastricht University Medical Center+, 6229 HX Maastricht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ehsan","family":"Natour","sequence":"additional","affiliation":[{"name":"Department of Cardiothoracic Surgery, Maastricht University Medical Center+, 6229 HX Maastricht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pieter","family":"van Paassen","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Clinical Immunology, Maastricht University Medical Center+, 6229 HX Maastricht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samuel","family":"Heuts","sequence":"additional","affiliation":[{"name":"Department of Cardiothoracic Surgery, Maastricht University Medical Center+, 6229 HX Maastricht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,4,29]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"Lou, X., Leshnower, B.G., Binongo, J., Beckerman, Z., McPherson, L., and Chen, E.P. (Semin. Thorac. Cardiovasc. Surg., 2021). Re-Operative Aortic Arch Surgery in a Contemporary Series, Semin. Thorac. Cardiovasc. Surg., in press.","DOI":"10.1053\/j.semtcvs.2021.03.035"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"759","DOI":"10.1111\/bjh.12718","article-title":"How I Treat Thrombotic Thrombocytopenic Purpura and Atypical Haemolytic Uraemic Syndrome","volume":"164","author":"Scully","year":"2014","journal-title":"Br. J. Haematol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"258","DOI":"10.1093\/icvts\/ivab231","article-title":"Thrombotic microangiopathy following aortic surgery with hypothermic circulatory arrest: A single-centre experience of an underestimated cause of acute renal failure","volume":"34","author":"Kamla","year":"2021","journal-title":"Interact. Cardiovasc. Thorac. 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