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Nonspecific symptoms, heterogeneity within study populations, and variability among current diagnostic criteria contribute to misdiagnosis and underdiagnosis of this syndrome. Hematopoietic stem cell transplantation and associated risk factors precipitate endothelial injury, leading to HSCT-TMA and other endothelial injury syndromes such as hepatic veno-occlusive disease\/sinusoidal obstruction syndrome, idiopathic pneumonia syndrome, diffuse alveolar hemorrhage, capillary leak syndrome, and graft-versus-host disease. Endothelial injury can trigger activation of the complement system, promoting inflammation and the development of endothelial injury syndromes, ultimately leading to organ damage and failure. In particular, the lectin pathway of complement is activated by damage-associated molecular patterns (DAMPs) on the surface of injured endothelial cells. Pattern-recognition molecules such as mannose-binding lectin (MBL), collectins, and ficolins\u2014collectively termed lectins\u2014bind to DAMPs on injured host cells, forming activation complexes with MBL-associated serine proteases 1, 2, and 3 (MASP-1, MASP-2, and MASP-3). Activation of the lectin pathway may also trigger the coagulation cascade via MASP-2 cleavage of prothrombin to thrombin. Together, activation of complement and the coagulation cascade lead to a procoagulant state that may result in development of HSCT-TMA. Several complement inhibitors targeting various complement pathways are in clinical trials for the treatment of HSCT-TMA. In this article, we review the role of the complement system in HSCT-TMA pathogenesis, with a focus on the lectin pathway.<\/jats:p>","DOI":"10.1186\/s40164-021-00249-8","type":"journal-article","created":{"date-parts":[[2021,12,19]],"date-time":"2021-12-19T02:02:23Z","timestamp":1639879343000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":43,"title":["Role of the lectin pathway of complement in hematopoietic stem cell transplantation-associated endothelial injury and thrombotic microangiopathy"],"prefix":"10.1186","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8883-8208","authenticated-orcid":false,"given":"Eleni","family":"Gavriilaki","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent T.","family":"Ho","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wilhelm","family":"Schwaeble","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Dudler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohamed","family":"Daha","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Teizo","family":"Fujita","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sonata","family":"Jodele","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,12,19]]},"reference":[{"key":"249_CR1","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1007\/978-3-030-02278-5_1","volume-title":"The EBMT handbook: hematopoietic stem cell transplantation and cellular therapies","author":"R Storb","year":"2019","unstructured":"Storb R. HSCT: Historical Perspective. In: Carreras E, Dufour C, Mohty M, Kr\u00f6ger N, editors. The EBMT handbook: hematopoietic stem cell transplantation and cellular therapies. Cham (CH): Springer International Publishing; 2019. p. 3\u20139."},{"issue":"12","key":"249_CR2","doi-asserted-by":"crossref","first-page":"1495","DOI":"10.1038\/bmt.2011.65","volume":"46","author":"E Carreras","year":"2011","unstructured":"Carreras E, Diaz-Ricart M. The role of the endothelium in the short-term complications of hematopoietic SCT. Bone Marrow Transplant. 2011;46(12):1495\u2013502.","journal-title":"Bone Marrow Transplant"},{"issue":"15","key":"249_CR3","doi-asserted-by":"crossref","first-page":"2424","DOI":"10.1182\/bloodadvances.2019000143","volume":"3","author":"S Pagliuca","year":"2019","unstructured":"Pagliuca S, Michonneau D, de Fontbrune FS, del Galy AS, Xhaard A, Robin M, et al. Allogeneic reactivity-mediated endothelial cell complications after HSCT: a plea for consensual definitions. 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VTH has consulted for Alexion, Janssen, Jazz, and Omeros Corporation. WS has consulted for Omeros Corporation. TD is an employee of Omeros Corporation. MD and TF declare no conflicts of interest. SJ holds United States Patent No: US 10,815,296 B2, is conducting an NIH-funded study with the study drug provided by Alexion Pharmaceuticals at no charge to study subjects, and has received travel support and lecture honoraria from Omeros Corporation.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"57"}}