Progress in children′s central nerve system involved Langerhans cell histiocytosis
10.3760/cma.j.issn.1673-4408.2023.10.004
- VernacularTitle:中枢神经系统受累的儿童朗格汉斯细胞组织细胞增生症研究进展
- Author:
Yiting HOU
1
;
Yijin GAO
Author Information
1. 上海交通大学医学院附属上海儿童医学中心血液肿瘤科 200127
- Keywords:
Children;
Langerhans cell histiocytosis;
Central nerve system;
Neurodegeneration
- From:
International Journal of Pediatrics
2023;50(10):662-666
- CountryChina
- Language:Chinese
-
Abstract:
Langerhans cell histiocytosis (LCH) is a rare inflammatory myeloid neoplastic disease which is characterized by CD1a + /CD207 + dendritic cell proliferation.LCH can affect multiple systems, and the prevalence of central nervous system involved LCH (CNS-LCH) ranges from 3.4% to 57%.The pathogenesis of CNS-LCH remains unclear.CNS-LCH can be divided into the following: focal mass lesions and lesions associated with neurodegeneration (ND). The clinical manifestations of CNS-LCH vary greatly due to different involved organs.The hypothalamic pituitary-adrenal (HPA) axis is among the most commonly involved site in CNS-LCH with focal mass lesions, and HPA infiltration presents clinically as diabetes insipidus and deficiency of anterior pituitary hormone secretion.LCH-ND is a rare, long-term neurologic complication which can seriously affect patients′life quality.It is mainly characterized by neurological disorders and/or progressive imaging changes.The current standard treatment of CNS-LCH focal mass lesions is based on the Histiocyte Society LCHIII approach, while there is no established optimal therapy for patients who develop LCH-ND.The pathogenesis, clinical manifestations, diagnosis and treatment of CNS-LCH are briefly reviewed in this article in order to provide a reference for clinical diagnosis and treatment.
