Clinical features and prognosis of acute B lymphoblastic leukemia children carrying a TCF3: : PBX1 fusion gene
10.3760/cma.j.cn101070-20241105-00717
- VernacularTitle:伴TCF3：：PBX1融合基因阳性的儿童急性B淋巴细胞白血病的临床特征和预后
- Author:
Lulu HUANG
1
;
Yunyan HE
;
Yang LI
;
Danna LIN
;
Ning LIAO
;
Yayun LING
;
Lyuhong XU
;
Xinyu LI
;
Huirong MAI
;
Ying WANG
;
Wuqing WAN
;
Ying LIU
;
Yanlai TANG
;
Xiaoli ZHANG
;
Chuan TIAN
;
Xiaofeng LI
;
Qiwen CHEN
;
Xingjiang LONG
;
Liuhua LIAO
;
Qiaoru LI
;
Jianling CAI
;
Zijun ZHEN
;
Zhiguang LI
;
Keyan YANG
;
Qinlong ZHENG
;
Lihua YANG
Author Information
1. 南方医科大学珠江医院小儿血液科，广州 510280
- Publication Type:Journal Article
- Keywords:
Acute lymphoblastic leukemia;
Child;
TCF3: : PBX1 fusion gene;
Prognosis
- From:
Chinese Journal of Applied Clinical Pediatrics
2025;40(7):497-502
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To analyze the clinical features and prognosis of acute B lymphoblastic leukemia (B-ALL) children carrying a TCF3: : PBX1 fusion gene and to evaluate the prognostic value of this gene.Methods:Retrospective cohort study.A total of 2 164 B-ALL children aged 0-18 years diagnosed and treated at 19 pediatric centers from October 2016 to June 2022 were enrolled.They were divided into the positive group and the negative group according to whether they carried a TCF3: : PBX1 fusion gene.The clinical characteristics, treatment response, adverse reactions, and prognosis of the 2 groups of patients were analyzed.The rank sum and Kruskal-Wallis tests were used to compare two and more than two groups of numerical variables, respectively.Fisher′s exact test was used to compare categorical variables.Results:Among the 2 164 patients, 116 (5.4%) were TCF3: : PBX1 positive, of which 70 patients were female, accounting for 60.3%.There were 840 female patients in the TCF3: : PBX1-negative group, accounting for 41.0%.There was a significant difference in the ratio of females between the TCF3: : PBX1-positive and TCF3: : PBX1-negative groups ( P<0.001).No significant difference was observed in age of onset between the two groups( P>0.05).The proportion of bone marrow naive cells [54.00 (14.00, 76.50)% vs.29.00 (3.00, 68.00)%], white blood cell counts [25.30 (10.46, 60.94)×10 9/L vs.9.03 (4.38, 30.73)×10 9/L] and hemoglobin counts [82.00(63.00, 101.00) g/L vs.74.00(60.00, 90.00) g/L] in the TCF3: : PBX1-positive group were significantly higher than those in the negative group at the onset (all P<0.05).In terms of treatment response, the proportion of peripheral blood naive cells on Day 8 in the TCF3: : PBX1-positive group was significantly higher than that in the negative group [2.00 (0, 9.00)% vs.0 (0, 2.00)%, P<0.001].The proportion of minimal residual disease <0.1% on Day 15 in the TCF3: : PBX1-positive group was significantly higher than that in the negative group ( P=0.038).There were no significant differences in cumulative recurrence rate, treatment-related mortality (TRM), and overall survival (OS) between the TCF3: : PBX1-positive group and TCF3: : PBX1-negative group (all P>0.05).The cumulative recurrence risk of TCF3: : PBX1-positive patients was 9.646 times higher than that of ETV6: : RUNX1-positive patients with better prognosis( HR=9.646, 95% CI: 1.026-90.700, P=0.047).There were no significant differences in TRM and OS between TCF3: : PBX1-positive and ETV6: : RUNX1-positive patients (all P>0.05).A significant enrichment of PAX5 mutations was detected in TCF3: : PBX1-positive patients.Among the 7 high-risk TCF3: : PBX1-positive patients in a single center, 4 patients had PAX5 mutations, and this proportion was significantly higher than that in other patients ( P<0.001). Conclusions:B-ALL children carrying a TCF3: : PBX1 fusion gene have a high remission rate and good long-term prognosis after intensive chemotherapy.It is suggesting that TCF3: : PBX1-positive B-ALL patients should be rated at intermediate risk to receive intensive chemotherapy.
