{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T19:15:07Z","timestamp":1786994107496,"version":"build-2736575974"},"reference-count":48,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"8","license":[{"start":{"date-parts":[[2021,7,14]],"date-time":"2021-07-14T00:00:00Z","timestamp":1626220800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2021,7,14]],"date-time":"2021-07-14T00:00:00Z","timestamp":1626220800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":["Liver Transpl"],"published-print":{"date-parts":[[2021,8]]},"abstract":"<jats:p>Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder. Liver involvement is seen in 10.1% to 19.8% of patients with LCH and can lead to secondary sclerosing cholangitis requiring liver transplantation (LT). We describe the characteristics and outcomes of patients undergoing LT for LCH. All patients undergoing a first LT for LCH in the United States were identified in the Scientific Registry of Transplant Recipients (SRTR) database (1987\u20102018). The Kaplan\u2010Meier curve method and log\u2010rank tests evaluated post\u2010LT survival. A systematic literature review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta\u2010analysis (PRISMA) statement. A total of 60 LCH LT recipients were identified in the SRTR, and 55 patients (91.7%) were children with median total bilirubin levels at LT of 5.8\u00a0mg\/dL (interquartile range [IQR], 2.7\u201012.9). A total of 49 patients (81.7%) underwent deceased donor LT (DDLT). The 1\u2010year, 3\u2010year, and 5\u2010year patient survival rates were 86.6%, 82.4%, and 82.4%, respectively. The systematic review yielded 26 articles reporting on 50 patients. Of the patients, 41 were children (82.0%), 90.0% had multisystem LCH, and most patients underwent DDLT (91.9%; n\u00a0=\u00a034\/37). Pre\u2010LT chemotherapy was administered in 74.0% and steroids in 71.7% (n\u00a0=\u00a033\/46) of the patients, and a recurrence of LCH to the liver was reported in 8.0% of the patients. Of the 50 patients, 11 (22.0%) died during a median follow\u2010up of 25.2\u00a0months (IQR, 9.0\u201051.6), and the 1\u2010year patient survival rate was 79.4%. LT can be considered as a feasible life\u2010saving option for the management of liver failure secondary to LCH in well\u2010selected patients.<\/jats:p>","DOI":"10.1002\/lt.25995","type":"journal-article","created":{"date-parts":[[2021,1,23]],"date-time":"2021-01-23T15:20:22Z","timestamp":1611415222000},"page":"1181-1190","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":14,"title":["Liver Transplantation for Langerhans Cell Histiocytosis: A US Population\u2010Based Analysis and Systematic Review of the Literature"],"prefix":"10.1097","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6742-6909","authenticated-orcid":false,"given":"Ioannis A.","family":"Ziogas","sequence":"first","affiliation":[{"name":"Department of Surgery,Division of Hepatobiliary Surgery and Liver Transplantation,Vanderbilt University Medical Center,Nashville,TN"},{"name":"Surgery Working Group,Society of Junior Doctors,Athens,Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christos D.","family":"Kakos","sequence":"additional","affiliation":[{"name":"Surgery Working Group,Society of Junior Doctors,Athens,Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"W. Kelly","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Surgery,Division of Hepatobiliary Surgery and Liver Transplantation,Vanderbilt University Medical Center,Nashville,TN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin I.","family":"Montenovo","sequence":"additional","affiliation":[{"name":"Department of Surgery,Division of Hepatobiliary Surgery and Liver Transplantation,Vanderbilt University Medical Center,Nashville,TN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lea K.","family":"Matsuoka","sequence":"additional","affiliation":[{"name":"Department of Surgery,Division of Hepatobiliary Surgery and Liver Transplantation,Vanderbilt University Medical Center,Nashville,TN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Zarnegar\u2010Lumley","sequence":"additional","affiliation":[{"name":"Department of Pediatrics,Division of Hematology\/Oncology,Vanderbilt University Medical Center,Nashville,TN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophoclis P.","family":"Alexopoulos","sequence":"additional","affiliation":[{"name":"Department of Surgery,Division of Hepatobiliary Surgery and Liver Transplantation,Vanderbilt University Medical Center,Nashville,TN"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2021,7,14]]},"reference":[{"key":"lt25995-bib-0001-20250824","doi-asserted-by":"crossref","first-page":"e491","DOI":"10.1097\/MPH.0000000000001662","article-title":"A case of Langerhans cell histiocytosis with multifocal, single\u2010system GI tract involvement and literature review","volume":"42","year":"2020","journal-title":"J Pediatr Hematol Oncol"},{"key":"lt25995-bib-0002-20250824","doi-asserted-by":"crossref","first-page":"856","DOI":"10.1056\/NEJMra1607548","article-title":"Langerhans\u2010cell histiocytosis","volume":"379","year":"2018","journal-title":"N Engl J Med"},{"key":"lt25995-bib-0003-20250824","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1002\/pbc.21498","article-title":"Descriptive epidemiology of childhood Langerhans cell histiocytosis in France, 2000\u20102004","volume":"51","year":"2008","journal-title":"Pediatr Blood Cancer"},{"key":"lt25995-bib-0004-20250824","doi-asserted-by":"crossref","first-page":"2341","DOI":"10.1016\/S0959-8049(03)00672-5","article-title":"Langerhans cell histiocytosis in adults. 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