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- Title
Long-term outcome, clinical course and treatment approaches of paediatric langerhans cell histiocytosis: A greek reference centre report.
- Authors
Tzotzola, Vasiliki; Petrikkos, Loizos; Papadakis, Vassilios; Mitropoulou, Georgia; Kelaidi, Charikleia; Dimitriadis, Efthymios; Polychronopoulou, Sophia
- Abstract
<bold>Aim: </bold>Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia with diverse clinical behaviour. In this article, we studied the clinical course, management and long-term outcomes of a paediatric cohort treated by our reference centre.<bold>Methods: </bold>We retrospectively studied 66 children with LCH, consecutively diagnosed by a Greek reference centre from 1974 to 2020.<bold>Results: </bold>The patients had a median age of 3.9 (range 0.0-15.9) years, 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-up period of 4.1 (range 0.5-27.7) years. The 10-year event-free survival and overall survival were 65.0% and 90.3% and improved significantly over a 45-year 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.<bold>Conclusion: </bold>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.
- Subjects
LANGERHANS-cell histiocytosis; OVERALL survival; PROGNOSIS; SURVIVAL rate; DIABETES insipidus; BRAF genes
- Publication
Acta Paediatrica, 2021, Vol 110, Issue 6, p1944
- ISSN
0803-5253
- Publication type
journal article
- DOI
10.1111/apa.15743