{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T02:35:29Z","timestamp":1791858929220,"version":"4.3.4"},"reference-count":12,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2026,1,17]],"date-time":"2026-01-17T00:00:00Z","timestamp":1768608000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,1,17]],"date-time":"2026-01-17T00:00:00Z","timestamp":1768608000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Ann Hematol"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Transplant-associated thrombotic microangiopathy (TA-TMA) is a fatal complication associated with hematopoietic stem cell transplant (HSCT). Endothelial dysfunction and complement activation cause consumptive thrombocytopenia with intravascular hemolysis, resulting in end-organ damage, especially to the kidneys and lungs. There are no U.S. Food and Drug Administration (FDA)-approved agents for TA-TMA, although Eculizumab is the most commonly used agent to treat TA-TMA. Patients who do not respond to Eculizumab have a dismal prognosis, with reported mortality up to 80%. Narsoplimab, a mannan-binding lectin-associated serine protease-2 (MASP-2) inhibitor, has been shown to treat TA-TMA by inhibiting the lectin pathway of the complement cascade. We report the first adult case with successful management of eculizumab-refractory TA-TMA with Narsoplimab. Our patient received a matched unrelated donor (bone marrow) allogenic HSCT and subsequently developed multi-organ damage. He was refractory to numerous treatments, including eculizumab, steroids, rituximab, and plasma exchange. After developing diffuse Alveolar hemorrphage and renal failure, he was initiated on Narsoplimab and later achieved a complete hematological response and became transfusion independent. This case highlights the importance of early recognition of TA-TMA and the need to switch therapy to other complement inhibitors if resistance to Eculizumab is noted.<\/jats:p>","DOI":"10.1007\/s00277-026-06756-0","type":"journal-article","created":{"date-parts":[[2026,1,17]],"date-time":"2026-01-17T03:27:22Z","timestamp":1768620442000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Use of narsoplimab for eculizumab-refractory adult transplant-associated thrombotic microangiopathy (TA-TMA)"],"prefix":"10.1007","volume":"105","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8749-8523","authenticated-orcid":false,"given":"Sara","family":"Young","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8737-4053","authenticated-orcid":false,"given":"Indumathy","family":"Varadarajan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,1,17]]},"reference":[{"issue":"11","key":"6756_CR1","doi-asserted-by":"publisher","first-page":"2344","DOI":"10.1016\/j.bbmt.2018.05.010","volume":"24","author":"M Postalcioglu","year":"2018","unstructured":"Postalcioglu M, Kim HT, Obut F et al (2018) Impact of thrombotic microangiopathy on renal outcomes and survival after hematopoietic stem cell transplantation. Biol Blood Marrow Transplant 24(11):2344\u20132353. https:\/\/doi.org\/10.1016\/j.bbmt.2018.05.010","journal-title":"Biol Blood Marrow Transplant"},{"issue":"9","key":"6756_CR2","doi-asserted-by":"publisher","DOI":"10.1111\/ctr.13371","volume":"32","author":"E Gavriilaki","year":"2018","unstructured":"Gavriilaki E, Sakellari I, Batsis I et al (2018) Transplant-associated thrombotic microangiopathy: incidence, prognostic factors, morbidity, and mortality in allogeneic hematopoietic cell transplantation. Clin Transplant 32(9):e13371. https:\/\/doi.org\/10.1111\/ctr.13371","journal-title":"Clin Transplant"},{"issue":"3","key":"6756_CR3","doi-asserted-by":"publisher","first-page":"151","DOI":"10.1016\/j.jtct.2022.11.015","volume":"29","author":"ML Schoettler","year":"2023","unstructured":"Schoettler ML, Carreras E, Cho B et al (2023) Harmonizing definitions for diagnostic criteria and prognostic assessment of transplantation-associated thrombotic microangiopathy: a report on behalf of the European Society for Blood and Marrow Transplantation, American Society for Transplantation and Cellular Therapy, Asia-Pacific Blood and Marrow Transplantation Group, and Center for International Blood and Marrow Transplant Research. Transplant Cell Ther 29(3):151\u2013163. https:\/\/doi.org\/10.1016\/j.jtct.2022.11.015","journal-title":"Transplant Cell Ther"},{"key":"6756_CR4","doi-asserted-by":"publisher","DOI":"10.3389\/fped.2019.00133","volume":"7","author":"CC Dvorak","year":"2019","unstructured":"Dvorak CC, Higham C, Shimano KA (2019) Transplant-associated thrombotic microangiopathy in pediatric hematopoietic cell transplant recipients: a practical approach to diagnosis and management. Front Pediatr 7:133. https:\/\/doi.org\/10.3389\/fped.2019.00133","journal-title":"Front Pediatr"},{"issue":"8","key":"6756_CR5","doi-asserted-by":"publisher","first-page":"1805","DOI":"10.1038\/s41409-021-01283-0","volume":"56","author":"JA Young","year":"2021","unstructured":"Young JA, Pallas CR, Knovich MA (2021) Transplant-associated thrombotic microangiopathy: theoretical considerations and a practical approach to an unrefined diagnosis. Bone Marrow Transplant 56(8):1805\u20131817. https:\/\/doi.org\/10.1038\/s41409-021-01283-0","journal-title":"Bone Marrow Transplant"},{"issue":"3","key":"6756_CR6","doi-asserted-by":"publisher","first-page":"191","DOI":"10.1016\/j.blre.2014.11.001","volume":"29","author":"S Jodele","year":"2015","unstructured":"Jodele S, Laskin BL, Dandoy CE et al (2015) A new paradigm: diagnosis and management of HSCT-associated thrombotic microangiopathy as multi-system endothelial injury. Blood Rev 29(3):191\u2013204. https:\/\/doi.org\/10.1016\/j.blre.2014.11.001","journal-title":"Blood Rev"},{"issue":"13","key":"6756_CR7","doi-asserted-by":"publisher","first-page":"1049","DOI":"10.1182\/blood.2019004218","volume":"135","author":"S Jodele","year":"2020","unstructured":"Jodele S, Dandoy CE, Lane A et al (2020) Complement blockade for TA-TMA: lessons learned from a large pediatric cohort treated with eculizumab. Blood 135(13):1049\u20131057. https:\/\/doi.org\/10.1182\/blood.2019004218","journal-title":"Blood"},{"issue":"12","key":"6756_CR8","doi-asserted-by":"publisher","first-page":"1112","DOI":"10.1182\/blood.2023022526","volume":"143","author":"S Jodele","year":"2024","unstructured":"Jodele S, Dandoy CE, Aguayo-Hiraldo P et al (2024) A prospective multi-institutional study of eculizumab to treat high-risk stem cell transplantation-associated TMA. Blood 143(12):1112\u20131123. https:\/\/doi.org\/10.1182\/blood.2023022526","journal-title":"Blood"},{"issue":"8","key":"6756_CR9","doi-asserted-by":"publisher","first-page":"1161","DOI":"10.1038\/s41409-024-02305-3","volume":"59","author":"M Castelli","year":"2024","unstructured":"Castelli M, Mic\u00f2 MC, Grassi A et al (2024) Safety and efficacy of narsoplimab in pediatric and adult patients with transplant-associated thrombotic microangiopathy: a real-world experience. Bone Marrow Transplant 59(8):1161\u20131168. https:\/\/doi.org\/10.1038\/s41409-024-02305-3","journal-title":"Bone Marrow Transplant"},{"issue":"22","key":"6756_CR10","doi-asserted-by":"publisher","first-page":"2447","DOI":"10.1200\/JCO.21.02389","volume":"40","author":"SK Khaled","year":"2022","unstructured":"Khaled SK, Claes K, Goh YT et al (2022) Narsoplimab, a mannan-binding lectin-associated serine protease-2 inhibitor, for the treatment of adult hematopoietic stem-cell transplantation-associated thrombotic microangiopathy. J Clin Oncol 40(22):2447\u20132457. https:\/\/doi.org\/10.1200\/JCO.21.02389","journal-title":"J Clin Oncol"},{"issue":"3","key":"6756_CR11","doi-asserted-by":"publisher","first-page":"336.e1","DOI":"10.1016\/j.jtct.2023.12.017","volume":"30","author":"ML Schoettler","year":"2024","unstructured":"Schoettler ML, Patel S, Bryson E et al (2024) Compassionate use narsoplimab for severe refractory transplantation-associated thrombotic microangiopathy in children. 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Bone Marrow Transplant 59(6):904\u2013906. https:\/\/doi.org\/10.1038\/s41409-024-02263-w","DOI":"10.1038\/s41409-024-02263-w"}],"container-title":["Annals of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00277-026-06756-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00277-026-06756-0","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00277-026-06756-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,5]],"date-time":"2026-02-05T03:33:17Z","timestamp":1770262397000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00277-026-06756-0"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,1,17]]},"references-count":12,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2026,1]]}},"alternative-id":["6756"],"URL":"https:\/\/doi.org\/10.1007\/s00277-026-06756-0","relation":{"has-preprint":[{"id-type":"doi","id":"10.21203\/rs.3.rs-6940683\/v1","asserted-by":"object"}]},"ISSN":["1432-0584"],"issn-type":[{"value":"1432-0584","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,1,17]]},"assertion":[{"value":"20 June 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"15 December 2025","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"17 January 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Sara Young MD \u2013 does not have any relevant financial disclosure.Indumathy Varadarajan MBBS \u2013 received consulting fees from Omeros, Kadmon and Kite in the period of 2023\u20132025.Our institution does not require ethics approval for reporting individual cases or case series. Written informed consent was obtained for anonymized patient information to be published in this article.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}},{"value":"No patient identifying data has been used in this case report. The patient has consented to share their HPI, hospital course in this case report. Please review attached consent form.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent to publish"}}],"article-number":"28"}}