Pulmonary involvement in pediatric-onset multisystem Langerhans cell histiocytosis: effect on course and outcome.
Details
Serval ID
serval:BIB_906B0DA993A1
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Pulmonary involvement in pediatric-onset multisystem Langerhans cell histiocytosis: effect on course and outcome.
Journal
Journal of Pediatrics
Working group(s)
German Society for Pediatric Hematology
Contributor(s)
Dornbirn£££A Ö Krankenhaus£££ A. , Ausserer B., Urban C., Meister B., Kaulfersch W., Kerbl R., Schmidt K., Sperl W., Gadner H., Van Gool S., Frühwald M., Schweigerer L., Henze G., Otte J., Dilloo D., Eberl W., Pekrun A., Scholbach T., Erler T., Paulussen M., Schneider D., Suttorp M., Borkhardt A., Sauerbrey A., Holter W., Eggert A., Klingebiel T., Niemeyer C., Reiter A., Lakomek M., Lode H., Körholz D., Janka G., Reinhardt D., Witt O., Längler A., Graf N., Beck JF., Nathrath M., Schrappe M., Nüßlein T., Berthold F., Niehues T., Christiansen H., Herting E., Vorwerk P., Faber J., Dürken M., Zimmer KP., Burdach S., Klein C., Jürgens H., Scheurlen W., Müller H., Corbacioglu S., Wigger M., Bielack S., Rauh W., Handgretinger R., Debatin K-., Schlegel PG., Kühne T., Leibundgut K., Ozsahin H., Beck-Popovic M., Neuhaus T., Niggli F., Kinderziekenhuis E., van den Bos C., Kamps WA., Bökkering JP., Pieters R.
ISSN
1097-6833 (Electronic)
ISSN-L
0022-3476
Publication state
Published
Issued date
07/2012
Peer-reviewed
Oui
Volume
161
Number
1
Pages
129-33.e1-3
Language
english
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov't
Publication Status: ppublish
Publication Status: ppublish
Abstract
OBJECTIVES: 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.
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.
Keywords
Adolescent, Child, Child, Preschool, Female, Histiocytosis, Langerhans-Cell/complications, Humans, Infant, Infant, Newborn, Lung Diseases/etiology, Male, Prognosis, Retrospective Studies
Pubmed
Create date
17/10/2014 11:01
Last modification date
20/08/2019 15:53
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