Analysis of 43 cases of Langerhans cell histiocytosis (LCH)-induced central diabetes insipidus registered in the JLSG-96 and JLSG-02 studies in Japan

Yoko Shioda, Souichi Adachi, Shinsaku Imashuku, Kazuko Kudo, Toshihiko Imamura, Akira Morimoto

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

To determine the ability of recent systemic chemotherapy protocols to reduce the incidence of central diabetes insipidus (CDI) in Langerhans cell histiocytosis (LCH), 43 CDI cases that belonged to a cohort of 348 pediatric patients with multi-focal LCH who were treated with the JLSG-96/-02 protocols were analyzed. The overall incidence of CDI was 12.4%, but in 24 cases CDI was already present at the time LCH was diagnosed. Thus, CDI developed during or after systemic chemotherapy over a follow-up period of 5.0 (0.2-14.7) years in only 19 patients (5.9%), with 7.4% at 5-year cumulative risk by Kaplan-Meier analysis. In two cases, complete resolution of CDI was noted. Anterior pituitary hormone deficiency was detected in 13 cases, while CDI-associated neurodegenerative disease was observed in six cases. The JLSG-96/-02 protocol appears to effectively reduce the occurrence of CDI. However, novel therapeutic measures are required to reverse pre-existing CDI and to prevent CDI-associated neurological complications.

Original languageEnglish
Pages (from-to)545-551
Number of pages7
JournalInternational Journal of Hematology
Volume94
Issue number6
DOIs
Publication statusPublished - 01-12-2011
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Hematology

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