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.
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Authors | Yoko Shioda, Souichi Adachi, Shinsaku Imashuku, Kazuko Kudo, Toshihiko Imamura, Akira Morimoto |
Journal | International journal of hematology
(Int J Hematol)
Vol. 94
Issue 6
Pg. 545-51
(Dec 2011)
ISSN: 1865-3774 [Electronic] Japan |
PMID | 22139588
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Pituitary Hormones, Anterior
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Topics |
- Adolescent
- Brain
(pathology)
- Child
- Child, Preschool
- Diabetes Insipidus, Neurogenic
(diagnosis, epidemiology, etiology)
- Drug Therapy, Combination
- Female
- Histiocytosis, Langerhans-Cell
(complications, diagnosis, drug therapy)
- Humans
- Incidence
- Infant
- Kaplan-Meier Estimate
- Magnetic Resonance Imaging
- Male
- Neurodegenerative Diseases
(complications)
- Pituitary Hormones, Anterior
(deficiency)
- Treatment Outcome
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