Clinical analysis of 112 patients with single-system Langerhans cell histiocytosis
10.3760/cma.j.issn.2095-428X.2016.15.014
- VernacularTitle:单器官受累朗格罕细胞组织细胞增生症112例
- Author:
Li ZHANG
;
Hongyun LIAN
;
Honghao MA
;
Dong WANG
;
Chao GAO
;
Tianyou WANG
;
Rui ZHANG
- Publication Type:Journal Article
- Keywords:
Langerhans cell histiocytosis;
Single system;
Treatment
- From:
Chinese Journal of Applied Clinical Pediatrics
2016;31(15):1172-1174
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate the clinical characteristics,treatment and prognosis of single-system Langerhans cell histiocytosis (LCH) in children.Methods A retrospective analysis was performed in single-system LCH patients registered between January 2006 and December 2012 in Beijing Children＇s Hospital Affiliated to Capital Medical University.The patients were divided into 2 groups:the bone involvement group and the other organ involvement group.The patients were assessed at 5 weeks,11 weeks,25 weeks,3 months,6 months,1 year and 3 years.The data were analyzed by using SPSS 17.0 software.Results A total of 112 patients (66 boys and 4,6 girls) with a median age of 5 years at diagnosis of LCH were analyzed.The most frequently affected organ was the bones(91 cases,81.3％),followed by skin(15 cases,13.4％).Few patients (27.6％) had acentral nervous system risk lesion,who were younger than those with other bone lesion(2.5 years vs 6.6 years).Patients with bone lesions were diagnosed at a significantly older age than other patients(5.6 years vs 1.5 years) (P ＜ 0.01).All patients received chemotherapy that included Prednisone and Vinblastine for 25 weeks.Twenty-five patients (22.3 ％) showed reactivation.Of these,4 patients exhibited reactivation in the pituitary.Three-year overall survival rate was expected to reach 100％,and no-event survival was expected at (73.22 ± 4.47) ％.Age of less than 2 years old was the factor of reactivation (P =0.033);sex,organ involvement and member of bone involved were not related with reactivation (P =0.679,0.142,0.639).Conclusions The bones were the frequent involvement organ in single-system LCH patients.These patients have a good prognosis.The rate of reactivation of single system-LCH can be decreased by chemotherapy.
