{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T01:35:54Z","timestamp":1792546554908,"version":"4.5.0"},"reference-count":36,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2023,6,26]],"date-time":"2023-06-26T00:00:00Z","timestamp":1687737600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Cancer Science"],"published-print":{"date-parts":[[2023,9]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Langerhans cell histiocytosis (LCH) is a rare disease characterized by clonal expansion of CD1a\n                    <jats:sup>+<\/jats:sup>\n                    CD207\n                    <jats:sup>+<\/jats:sup>\n                    myeloid dendritic cells. The features of LCH are mainly described in children and remain poorly defined in adults; therefore, we conducted a nationwide survey to collect clinical data from 148 adult patients with LCH. The median age at diagnosis was 46.5 (range: 20\u201387) years with male predominance (60.8%). Among the 86 patients with detailed treatment information, 40 (46.5%) had single system LCH, whereas 46 (53.5%) had multisystem LCH. Moreover, 19 patients (22.1%) had an additional malignancy.\n                    <jats:italic>BRAF<\/jats:italic>\n                    V600E in plasma cell\u2010free DNA was associated with a low overall survival (OS) rate and the risk of the pituitary gland and central nervous system involvement. At a median follow\u2010up of 55\u2009months from diagnosis, six patients (7.0%) had died, and the four patients with LCH\u2010related death did not respond to initial chemotherapy. The OS probability at 5\u2009years post\u2010diagnosis was 90.6% (95% confidence interval: 79.8\u201395.8). Multivariate analysis showed that patients aged \u226560\u2009years at diagnosis had a relatively poor prognosis. The probability of event\u2010free survival at 5\u2009years was 52.1% (95% confidence interval: 36.6\u201365.5), with 57 patients requiring chemotherapy. In this study, we first revealed the high rate of relapse after chemotherapy and mortality of poor responders in adults as well as children. Therefore, prospective therapeutic studies of adults with LCH using targeted therapies are needed to improve outcomes in adults with LCH.\n                  <\/jats:p>","DOI":"10.1111\/cas.15879","type":"journal-article","created":{"date-parts":[[2023,6,26]],"date-time":"2023-06-26T18:49:06Z","timestamp":1687805346000},"page":"3687-3697","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":21,"title":["Clinical and prognostic features of Langerhans cell histiocytosis in adults"],"prefix":"10.1111","volume":"114","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1714-1347","authenticated-orcid":false,"given":"Aki","family":"Sato","sequence":"first","affiliation":[{"name":"Department of Hematology and Oncology, Institute of Medical Science Research Hospital University of Tokyo  Tokyo 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