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We report the first known case of the use of factor B inhibition in a 21-year-old male with complement-mediated thrombotic microangiopathy (CMTMA), later found to have Castleman\u2019s disease as a mimicker of aHUS.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case presentation<\/jats:title>\n                    <jats:p>Initially, the patient partially responded to eculizumab and then fully to ravulizumab. However, two years later, he experienced a flare that did not improve with ravulizumab redosing. An application for the compassionate use of Iptacopan was approved by the United States Food and Drug Administration, based on its success in C5 blockade Paroxysmal Nocturnal Hemoglobinuria. The patient\u2019s blood counts recovered, his microangiopathic hemolytic anemia ceased, and his acute kidney injury resolved allowing cessation of renal replacement therapy. Human Anti-murine anti-drug antibodies were suspected as the cause of C5 blockade failure but were unconfirmed. Ten months later, the patient\u2019s aHUS relapsed and he was concurrently diagnosed with Castleman\u2019s syndrome, a mimicker of aHUS, prompting the initiation of dual combination therapy of C5 blockade inhibitor and Factor B inhibitor and siltuximab.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>This case marks the first use of Iptacopan for aHUS\/CMTMA and the first use of dual combination therapy of eculizumab and Factor B Inhibition for patients with aHUS\/CMTMA and Castleman\u2019s disease in the United States at the University of California Irvine by our thrombotic microangiopathy assessment team.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Clinical trial<\/jats:title>\n                    <jats:p>N\/A, this study is not a clinical trial.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s12882-025-04487-4","type":"journal-article","created":{"date-parts":[[2025,10,24]],"date-time":"2025-10-24T09:53:50Z","timestamp":1761299630000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Iptacopan in C5 blockade refractory atypical hemolytic uremic syndrome with associated Castleman\u2019s disease: case report"],"prefix":"10.1186","volume":"26","author":[{"given":"Matthew D.","family":"Nguyen","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefan","family":"Ciurea","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sheetal","family":"Desai","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arash Rezazadeh","family":"Kalebasty","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Minh-Ha","family":"Tran","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Caroline","family":"Gee","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mina","family":"Tadros","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vu Q.","family":"Nguyen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Samir","family":"Patel","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Umut","family":"Selamet","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ramy","family":"Hanna","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2025,10,24]]},"reference":[{"issue":"2","key":"4487_CR1","doi-asserted-by":"publisher","first-page":"149","DOI":"10.1053\/j.ackd.2021.11.006","volume":"29","author":"RM Hanna","year":"2022","unstructured":"Hanna RM, Henriksen K, Kalantar-Zadeh K, Ferrey A, Burwick R, Jhaveri KD. 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He also serves as a consultant and advisor for Novartis, Alexion, and Calliditas. He serves on the speaker\u2019s bureau for Otsuka, Alexion, Calliditas, Aurinia, GSK, and Calliditas. SC served as advisory board member for Hansa Biopharma, CareDx, Spectrum\/Achrotech, Kiadis Pharma, Allogene Celularity, MolMed, Pharmacyclics and received research funds from Kiadis and Miltenyi Biotech. M. Tran received an honoraria from Sanofi. ARK owns stock and ownership interests in ECOM Medical and has received honoraria from companies such as Exelixis, Bayer, Eisai, Aveo, and AstraZeneca. Additionally, he has served on the Speakers\u2019 Bureau for Janssen, Pfizer, and Sanofi. ARK has also received travel, accommodations, and expenses from Janssen, Eisai, Bayer, and Exelixis. Furthermore, he has received research funding from numerous companies including Genentech, Exelixis, Janssen, AstraZeneca, Bayer, Bristol-Myers Squibb, and others.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"585"}}