Clinical features and prognosis of ETV6-RUNX1-positive childhood B-precursor acute lymphocyte leukemia
10.3760/cma.j.issn.0253-2727.2021.01.009
- VernacularTitle:ETV6-RUNX1融合基因阳性儿童急性前体B淋巴细胞白血病的临床特征及预后分析
- Author:
Yongzhi ZHENG
1
;
Lili PAN
;
Jian LI
;
Zaisheng CHEN
;
Xueling HUA
;
Shaohua LE
;
Hao ZHENG
;
Cai CHEN
;
Jianda HU
Author Information
1. 福建医科大学附属协和医院小儿血液科，福州 350001
- Keywords:
ETV6-RUNX1 fusion gene;
Leukemia, lymphoblastic, acute;
Child;
Prognosis
- From:
Chinese Journal of Hematology
2021;42(1):45-51
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinical features and prognosis of ETV6-RUNX1-positive childhood B-precursor acute lymphocyte leukemia (B-ALL) .Methods:The clinical data of 927 newly diagnosed children with B-ALL admitted to the Fujian Medical University Union Hospital from April 2011 to May 2020 were retrospectively analyzed. According to the results of ETV6-RUNX1 gene, the patients were divided into ETV6-RUNX1 + and ETV6-RUNX1 - groups. The clinical features and prognosis between the two groups were compared. Among the 182 children with ETV6-RUNX1 +, 144 patients received the Chinese Childhood Leukemia Collaborative Group (CCLG) -ALL 2008 protocol (CCLG-ALL 2008 group) and 38 received the China Childhood Cancer Collaborative Group (CCCG) -ALL2015 protocol (CCCG-ALL 2015 group) . The efficacy, serious adverse effects (SAE) incidence, and treatment-related mortality (TRM) of the two groups were also compared. Results:Of the 927 B-ALL patients, 189 (20.4% ) were ETV6-RUNX1 +. The proportion of patients with risk factors (age ≥10 years or <1 year, white blood cell count ≥50×10 9/L) in the ETV6-RUNX1 + group was significantly lower than that in the ETV6-RUNX1 - group ( P=0.000, 0.001, respectively) , while the proportion of patients with good early response (good response to prednisone, d15 or d19 MRD <1% , and d33 or d46 MRD<0.01% in induction chemotherapy) in the ETV6-RUNX1 + group was significantly higher than that in the ETV6-RUNX1 - group ( P=0.028, 0.004, respectively) . The 5-year EFS and OS of the ETV6-RUNX1 + group were significantly higher than those of the ETV6-RUNX1 - group (EFS: 89.8% vs 83.2% , P=0.003; OS: 90.2% vs 86.3% , P=0.030) . The incidence of infection-related SAE and TRM was significantly higher than that of CCCG-ALL 2015 group. A statistical difference was observed between the incidence of infection-related SAE of the two groups (27.1% vs 5.3% , P=0.004) , but no difference in TRM (4.9% vs 0, P=0.348) . Conclusion:ETV6-RUNX1 +B-ALL children have fewer risk factors at diagnosis, better early response, lower recurrence rate, and good prognosis than that of ETV6-RUNX1 -B-ALL children. Reducing the intensity of chemotherapy appropriately can lower the infection-related SAE and TRM and improve the long-term survival in this subtype.
