{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T17:57:39Z","timestamp":1781632659398,"version":"3.54.5"},"reference-count":20,"publisher":"Oxford University Press (OUP)","issue":"10","license":[{"start":{"date-parts":[[2024,7,27]],"date-time":"2024-07-27T00:00:00Z","timestamp":1722038400000},"content-version":"vor","delay-in-days":1,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"Beijing Natural Science Haidian","award":["L222081"],"award-info":[{"award-number":["L222081"]}]},{"name":"National Key R&D Program of China, Ministry of Science and Technology of the People\u2019s Republic of China","award":["2022YFC2304605"],"award-info":[{"award-number":["2022YFC2304605"]}]},{"name":"National High Level Hospital Clinical Research Funding","award":["2022-PUMCH-A-080"],"award-info":[{"award-number":["2022-PUMCH-A-080"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,10,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background and Aims<\/jats:title>\n                  <jats:p>Liver involvement portends poor prognosis in adults. We aimed to characterize the clinical features, liver function tests, radiologic findings, molecular profiles, therapeutic approaches and outcomes of adults patients with Langerhans cell histiocytosis (LCH) with liver involvement.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>We conducted a retrospective analysis of all adults with LCH (\u2265\u200518 years) seen at Peking Union Medical College Hospital (Beijing, China) between January 2001 and December 2022.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Among the 445 newly diagnosed adults with LCH, 90 patients had liver involvement at diagnosis and 22 patients at relapse. The median age was 32 years (range, 18\u201366 years). Of 112 evaluable patients, 108 had full liver function testing, including alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase (ALP), \u03b3-glutamyl transpeptidase (GGT), and total bilirubin and albumin. Elevated ALP was seen in 63.0% and GGT in 86.1%; 14.8% had elevated bilirubin. Next-generation sequencing of 54 patients revealed frequent BRAFN486_P490 (29.6%), BRAFV600E (18.5%), and MAP2K1 (14.8%).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Outcomes<\/jats:title>\n                  <jats:p>After a median 40 months\u2019 follow-up (range 1-168 months), 3-year progression-free survival (PFS) and overall survival were 49.7% and 86.6% respectively. In multivariable analyses, \u22653 abnormal liver function tests (HR 3.384, 95% CI 1.550\u20137.388, P\u2005=\u2005.002) associated with inferior PFS; immunomodulatory drug therapy (HR 0.073, 95% CI, 0.010-0.541, P\u2005=\u2005.010) correlated with superior PFS versus chemotherapy.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>In summary, elevated GGT and ALP were common in adults with LCH liver involvement. Greater than equal to 3 abnormal liver function tests predicted poor outcomes. Immunomodulatory drug therapy was associated with favorable progression-free survival compared to chemotherapy.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/oncolo\/oyae175","type":"journal-article","created":{"date-parts":[[2024,7,27]],"date-time":"2024-07-27T08:26:40Z","timestamp":1722068800000},"page":"e1347-e1353","source":"Crossref","is-referenced-by-count":7,"title":["Liver involvement with Langerhans cell histiocytosis in adults"],"prefix":"10.1093","volume":"29","author":[{"given":"Long","family":"Chang","sequence":"first","affiliation":[{"name":"Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College , Beijing ,","place":["People\u2019s Republic of China"]},{"name":"State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital , Beijing ,","place":["People\u2019s Republic of 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