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Long-term outcome, clinical course and treatment approaches of paediatric langerhans cell histiocytosis: A greek reference centre report.

Authors
  • Tzotzola, Vasiliki1
  • Petrikkos, Loizos1
  • Papadakis, Vassilios1
  • Mitropoulou, Georgia2
  • Kelaidi, Charikleia1
  • Dimitriadis, Efthymios3
  • Polychronopoulou, Sophia1
  • 1 Department of Pediatric Hematology-Oncology, "Aghia Sophia" Children's Hospital, Athens, Greece. , (Greece)
  • 2 Department of Pathology, "Aghia Sophia" Children's Hospital, Athens, Greece. , (Greece)
  • 3 Department of Genetics, "St. Savvas" Anticancer Hospital, Athens, Greece. , (Greece)
Type
Published Article
Journal
Acta Paediatrica
Publisher
Wiley (Blackwell Publishing)
Publication Date
Jun 01, 2021
Volume
110
Issue
6
Pages
1944–1951
Identifiers
DOI: 10.1111/apa.15743
PMID: 33382132
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

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. We retrospectively studied 66 children with LCH, consecutively diagnosed by a Greek reference centre from 1974 to 2020. 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. 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. © 2020 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.

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