Abstract | PURPOSE: METHODS: Retrospective data from 317 patients (multisystem disease (MS), n = 206; multiple focal bone (MFB), n = 111) treated according to the JLSG-96/02 regimens were analyzed. RESULTS: The median follow-up duration was 10.6 years (range, 0.1-21.1). A total of 50/317 (15.8%) patients developed CDI (MFB, n = 4; MS, n = 46). Of the 50 cases, CDI was already present at the time of LCH diagnosis (pre-CDI) in 25, and it newly developed after the diagnosis and initiation of treatment (post-CDI) in the other 25 cases. The cumulative incidence of post-CDI at 10-year calculated by Kaplan-Meier analysis was 9.0% for total and 12.0% for MS patients. A positive correlation with LCH lesions at the CNS risk sites at diagnosis was found in pre-CDI cases (17/164 vs 8/171; P = 0.0359), but not in post-CDI cases (14/129 vs 11/163; P = 0.254). Multivariate analysis showed that relapse at the CNS risk sites was significantly associated with post-CDI development (hazard ratio: 4.70; 95% CI, 1.29-17.1, P < 0.05). CONCLUSIONS: In the JLSG-96/02 studies, CDI developed in 15.8% of the cohort in which half as pre- and the other half as post-CDI. Relapse, particularly at the CNS risk sites, was linked with the development of post-CDI.
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Authors | Kenichi Sakamoto, Akira Morimoto, Yoko Shioda, Toshihiko Imamura, Shinsaku Imashuku, Japan LCH Study Group (JLSG) |
Journal | Pediatric blood & cancer
(Pediatr Blood Cancer)
Vol. 66
Issue 1
Pg. e27454
(01 2019)
ISSN: 1545-5017 [Electronic] United States |
PMID | 30207064
(Publication Type: Journal Article)
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Copyright | © 2018 Wiley Periodicals, Inc. |
Topics |
- Adolescent
- Child
- Child, Preschool
- Diabetes Insipidus, Neurogenic
(complications, physiopathology)
- Female
- Follow-Up Studies
- Histiocytosis, Langerhans-Cell
(epidemiology, pathology)
- Humans
- Incidence
- Infant
- Infant, Newborn
- Japan
(epidemiology)
- Male
- Prognosis
- Retrospective Studies
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