{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,14]],"date-time":"2025-05-14T00:40:38Z","timestamp":1747183238834,"version":"3.40.5"},"reference-count":26,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2021,1,10]],"date-time":"2021-01-10T00:00:00Z","timestamp":1610236800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acta Paediatrica"],"published-print":{"date-parts":[[2021,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aim<\/jats:title><jats:p>Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia with diverse clinical behaviour. In this article, we studied the clinical course, management and long\u2010term outcomes of a paediatric cohort treated by our reference centre.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We retrospectively studied 66 children with LCH, consecutively diagnosed by a Greek reference centre from 1974 to 2020.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The patients had a median age of 3.9 (range 0.0\u201315.9)\u00a0years, 39 and 6 patients were diagnosed with unifocal or multifocal single system disease and 14 and 7 had multisystem disease with or without risk organ involvement. No late occurrence of clinical neurodegenerative disease or diabetes insipidus were observed at a median follow\u2010up period of 4.1 (range 0.5\u201327.7) years. The 10\u2010year event\u2010free survival and overall survival were 65.0% and 90.3% and improved significantly over a 45\u2010year period. Survival was superior in single system than multisystem cases. BRAF V600E mutation was found in 8\/14 tested patients. Reactivation occurred in 12\/66 patients (18.2%); 11 achieved remission and one patient died after a second relapse.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>LCH survival rates significantly increased in our cohort over time. Reactivation occurred in 18.2% patients, but no late neurodegeneration was found. The prognostic value of single system disease status vs. multisystem LCH was confirmed.<\/jats:p><\/jats:sec>","DOI":"10.1111\/apa.15743","type":"journal-article","created":{"date-parts":[[2020,12,31]],"date-time":"2020-12-31T14:12:07Z","timestamp":1609423927000},"page":"1944-1951","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Long\u2010term outcome, clinical course and treatment approaches of paediatric langerhans cell histiocytosis: A greek reference centre report"],"prefix":"10.1111","volume":"110","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9828-8804","authenticated-orcid":false,"given":"Vasiliki","family":"Tzotzola","sequence":"first","affiliation":[{"name":"Department of Pediatric Hematology\u2010Oncology \u201cAghia Sophia\u201d Children\u2019s Hospital Athens 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