Clinical analysis of 72 children with Langerhans cell histiocytosis.
10.7499/j.issn.1008-8830.2412173
- Author:
Wen-Xuan JIANG
1
;
Fang-Hua YE
1
;
Yi-Xin XIAO
1
;
Wen-Jun DENG
1
;
Yan YU
1
;
Liang-Chun YANG
1
Author Information
1. Department of Pediatric Hematology, Xiangya Hospital of Central South University, Changsha 410008, China.
- Publication Type:Journal Article
- Keywords:
BRAF gene;
Child;
Clinical characteristics;
Langerhans cell histiocytosis;
Prognosis
- MeSH:
Humans;
Histiocytosis, Langerhans-Cell/therapy*;
Child, Preschool;
Child;
Male;
Infant;
Female;
Adolescent;
Retrospective Studies;
Proto-Oncogene Proteins B-raf/genetics*;
Prognosis;
Infant, Newborn;
Mutation
- From:
Chinese Journal of Contemporary Pediatrics
2025;27(5):555-562
- CountryChina
- Language:Chinese
-
Abstract:
OBJECTIVES:To study the clinical characteristics, efficacy, and prognosis of pediatric Langerhans cell histiocytosis (LCH).
METHODS:A retrospective analysis was conducted on 72 children with newly diagnosed LCH.
RESULTS:The median age of the 72 children was 5 years (range: 0-14 years), with skull involvement being the most common (56 cases, 77.8%). The BRAF-V600E mutation was not associated with clinical characteristics, efficacy, or prognosis (P>0.05). The 5-year overall survival rate was 91.6%±4.2%, and the 5-year event-free survival (EFS) rate was 67.5%±5.8%. The 6-week chemotherapy response rate and 5-year EFS rate were lower in the risk organ involvement group compared to the no risk organ involvement group (P<0.05). The five-year overall survival rates for the group with multi-system involvement and the group with platelet count ≥450×109/L were respectively lower than those for the single-system involvement group and the group with platelet count <450×109/L (P<0.05). Risk organ involvement is an independent risk factor for 5-year EFS (P<0.05).
CONCLUSIONS:Skull is the most commonly affected site in pediatric LCH. The BRAF-V600E mutation is not related to clinical characteristics, efficacy, or prognosis. Elevated platelet count, risk organ involvement, and multisystem involvement are associated with poor prognosis, with risk organ involvement being an independent risk factor for 5-year EFS.
