Analysis of 7 cases of childhood blastic plasmacytoid dendritic cell neoplasm
10.3760/cma.j.cn112140-20250215-00119
- VernacularTitle:儿童母细胞性浆细胞样树突细胞肿瘤7例分析
- Author:
Ziqing FENG
1
;
Chunju ZHOU
;
Ningning ZHANG
;
Ling JIN
;
Jing YANG
;
Shuang HUANG
;
Meng ZHANG
;
Nan LI
;
Yanlong DUAN
Author Information
1. 国家儿童医学中心 首都医科大学附属北京儿童医院儿童肿瘤中心肿瘤内科 儿童血液病与肿瘤分子分型北京市重点实验室 儿童肿瘤国家临床重点专科 儿科重大疾病研究教育部重点实验室，北京 100045
- Publication Type:Journal Article
- Keywords:
Child;
Neoplasms, plasma cell;
Dendritic cells;
Prognosis
- From:
Chinese Journal of Pediatrics
2025;63(11):1207-1211
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To evaluate the clinical characteristics, pathology, treatment and prognosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN) in children.Methods:Clinical data (including gender, age of disease onset, affected sites, treatment, timing of allogeneic hematopoietic stem cell transplantation (allo-HSCT), etc.) of 7 children with BPDCN who were admitted to Beijing Children′s Hospital, Capital Medical University from December 2018 to December 2023 were analyzed retrospectively. Clinical outcomes were also assessed, with patients followed up until December 2024.Results:Among 7 patients, there were 3 males and 4 females. Age at disease onset ranged from 3.2 to 12.9 years. Initial presentations included subcutaneous nodules in 5 cases, rash in 1 case, and ankle pain in 1 case. Extra-cutaneous involvement was seen in the bone marrow, lymph nodes, and central nervous system. Six patients received induction chemotherapy using a modified lymphoblastic lymphoma regimen, 1 patient received the high-risk protocol for pediatric lymphoblastic lymphoma/leukemia and salvage therapy regimens. Allo-HSCT was performed soon after chemotherapy remission. The time to bridge allo-HSCT was 3.5 to 6.5 months. The follow-up time was 1.6 to 6.0 years. Six patients were in disease-free survival, while 1 patient survived with disease after recurrence following transplantation.Conclusions:BPDCN is rare in children and presents diverse clinical manifestations, with skin involvement being the predominant feature. Early allo-HSCT following complete remission with chemotherapy can improve prognosis.
