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Ronceray, L.* ; Pötschger, U.* ; Janka, G.* ; Gadner, H.* ; Minkov, M.* ; German Society for Pediatric Hematology and Oncology (Nathrath, M.) ; Langerhans Cell Histiocytosis Study Group (*)

Pulmonary involvement in pediatric-onset multisystem Langerhans cell histiocytosis: Effect on course and outcome.

J. Pediatr. 161, 129-133 (2012)

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Open Access Green as soon as Postprint is submitted to ZB.

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To assess the effect of pulmonary involvement on the course and outcome of multisystem Langerhans cell histiocytosis (MS-LCH) in children. STUDY DESIGN: We conducted a retrospective analysis of 420 consecutive patients with MS-LCH. In this analysis, the term "risk organs" is defined as involvement of the liver, spleen, and/or hematopoietic system. The effect of pulmonary involvement on survival was assessed with multivariate Cox regression with adjustment for risk organs involvement and age. RESULTS: Pulmonary involvement in MS-LCH was present at diagnosis in 102 patients (24%). Of the 318 patients without pulmonary involvement at diagnosis, it developed in 28 within a median of 10 months (range, 1 month-5.5 years). The 5-year overall survival rate in patients without risk organ involvement at diagnosis was 96% in patients without pulmonary involvement and 94% in those with pulmonary involvement. In patients with risk organ involvement at diagnosis, the 5-year overall survival rate was 73% in patients without pulmonary involvement and 65% in patients with pulmonary involvement. In multivariate analysis, pulmonary involvement at diagnosis had no significant impact on survival rats (P = .109, hazard ratio = 1.5). CONCLUSIONS: In multivariate analysis, pulmonary involvement was not an independent prognostic variable and should therefore be excluded from the definition of risk organ involvement in MS-LCH.

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Publication type Article: Journal article

Document type Scientific Article

Corresponding Author

Keywords CHILDREN; THERAPY

ISSN (print) / ISBN 0022-3476

e-ISSN 1097-6833

Journal Journal of Pediatrics, The

Quellenangaben Volume: 161, Issue: 1, Pages: 129-133

Publisher Elsevier

Non-patent literature Publications

Reviewing status Peer reviewed

Institute(s) CCG Osteosarcoma (PATH-KOS)