Frontiers in Oncology (Dec 2021)
Prognostic Value and Outcome for ETV6/RUNX1-Positive Pediatric Acute Lymphoblastic Leukemia: A Report From the South China Children’s Leukemia Group
- Kun-yin Qiu,
- Kun-yin Qiu,
- Hong-gui Xu,
- Hong-gui Xu,
- Xue-qun Luo,
- Hui-rong Mai,
- Ning Liao,
- Li-hua Yang,
- Min-cui Zheng,
- Wu-qing Wan,
- Xue-dong Wu,
- Ri-yang Liu,
- Qi-wen Chen,
- Hui-qin Chen,
- Xiao-fei Sun,
- Hua Jiang,
- Xing-jiang Long,
- Guo-hua Chen,
- Xin-yu Li,
- Xin-yu Li,
- Chang-gang Li,
- Li-bin Huang,
- Ya-yun Ling,
- Dan-na Lin,
- Chuan Wen,
- Wen-yong Kuang,
- Xiao-qin Feng,
- Zhong-lv Ye,
- Bei-yan Wu,
- Xiang-lin He,
- Qiao-ru Li,
- Li-na Wang,
- Xian-ling Kong,
- Lu-hong Xu,
- Lu-hong Xu,
- Chi-kong Li,
- Jian-pei Fang,
- Jian-pei Fang
Affiliations
- Kun-yin Qiu
- Children’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
- Kun-yin Qiu
- Guangdong Provincial Key Laboratory of Malignant Tumor Epigenetics and Gene Regulation, Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University, Guangzhou, China
- Hong-gui Xu
- Children’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
- Hong-gui Xu
- Guangdong Provincial Key Laboratory of Malignant Tumor Epigenetics and Gene Regulation, Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University, Guangzhou, China
- Xue-qun Luo
- Department of Paediatrics, Sun Yat-sen University First Affiliated Hospital, Guangzhou, China
- Hui-rong Mai
- Department of Hematology and Oncology, Shenzhen Children’s Hospital, Shenzhen, China
- Ning Liao
- Department of Paediatrics, Guangxi Medical University First Affiliated Hospital, Nanning, China
- Li-hua Yang
- Department of Paediatrics, Southern Medical University Zhujiang Hospital, Guangzhou, China
- Min-cui Zheng
- Department of Hematology, Hunan Children’s Hospital, Changsha, China
- Wu-qing Wan
- Department of Paediatrics, Second Xiangya Hospital of Central South University, Changsha, China
- Xue-dong Wu
- Department of Paediatrics, Southern Medical University Nanfang Hospital, Guangzhou, China
- Ri-yang Liu
- 0Department of Paediatrics, Huizhou Central People’s Hospital, Huizhou, China
- Qi-wen Chen
- 1Department of Paediatrics, First Affiliated Hospital of Nanchang University, Nanchang, China
- Hui-qin Chen
- 2Department of Paediatrics, Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, China
- Xiao-fei Sun
- 3Department of Paediatrics, Sun Yat-sen University Cancer Center, Guangzhou, China
- Hua Jiang
- 4Department of Hematology, Guangzhou Women and Children’s Medical Center, Guangzhou, China
- Xing-jiang Long
- 5Department of Paediatrics, Liuzhou People’s Hospital, Liuzhou, China
- Guo-hua Chen
- 6Department of Paediatrics, Huizhou First People’s Hospital, HuiZhou, China
- Xin-yu Li
- Children’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
- Xin-yu Li
- Guangdong Provincial Key Laboratory of Malignant Tumor Epigenetics and Gene Regulation, Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University, Guangzhou, China
- Chang-gang Li
- Department of Hematology and Oncology, Shenzhen Children’s Hospital, Shenzhen, China
- Li-bin Huang
- Department of Paediatrics, Sun Yat-sen University First Affiliated Hospital, Guangzhou, China
- Ya-yun Ling
- Department of Paediatrics, Guangxi Medical University First Affiliated Hospital, Nanning, China
- Dan-na Lin
- Department of Paediatrics, Southern Medical University Zhujiang Hospital, Guangzhou, China
- Chuan Wen
- Department of Paediatrics, Second Xiangya Hospital of Central South University, Changsha, China
- Wen-yong Kuang
- Department of Hematology, Hunan Children’s Hospital, Changsha, China
- Xiao-qin Feng
- Department of Paediatrics, Southern Medical University Nanfang Hospital, Guangzhou, China
- Zhong-lv Ye
- 7Department of Paediatrics, Affiliated Hospital of Guangdong Medical University, Zhanjiang, China
- Bei-yan Wu
- 8Department of Paediatrics, The First Affiliated Hospital of Shantou University Medical College, Shantou, China
- Xiang-lin He
- 9Department of Paediatrics, Hunan Provincial People’s Hospital, Changsha, China
- Qiao-ru Li
- 0Department of Paediatrics, Zhongshan People’s Hospital, Zhongshan, China
- Li-na Wang
- 1Department of Paediatrics, Guangzhou First People’s Hospital, Guangzhou, China
- Xian-ling Kong
- 2Department of Paediatrics, Boai Hospital of Zhongshan, Zhongshan, China
- Lu-hong Xu
- Children’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
- Lu-hong Xu
- Guangdong Provincial Key Laboratory of Malignant Tumor Epigenetics and Gene Regulation, Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University, Guangzhou, China
- Chi-kong Li
- 3Department of Paediatrics, Hong Kong Children Hospital and Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong, Hong Kong SAR, China
- Jian-pei Fang
- Children’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
- Jian-pei Fang
- Guangdong Provincial Key Laboratory of Malignant Tumor Epigenetics and Gene Regulation, Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University, Guangzhou, China
- DOI
- https://doi.org/10.3389/fonc.2021.797194
- Journal volume & issue
-
Vol. 11
Abstract
PurposeTo analyzed the outcome of ETV6/RUNX1-positive pediatric acute B lymphoblastic leukemia (B-ALL) with the aim of identifying prognostic value.MethodA total of 2,530 pediatric patients who were diagnosed with B-ALL were classified into two groups based on the ETV6/RUNX1 status by using a retrospective cohort study method from February 28, 2008, to June 30, 2020, at 22 participating ALL centers.ResultsIn total, 461 (18.2%) cases were ETV6/RUNX1-positive. The proportion of patients with risk factors (age <1 year or ≥10 years, WB≥50×109/L) in ETV6/RUNX1-positive group was significantly lower than that in negative group (P<0.001), while the proportion of patients with good early response (good response to prednisone, D15 MRD < 0.1%, and D33 MRD < 0.01%) in ETV6/RUNX1-positive group was higher than that in the negative group (P<0.001, 0.788 and 0.004, respectively). Multivariate analysis of 2,530 patients found that age <1 or ≥10 years, SCCLG-ALL-2016 protocol, and MLL were independent predictor of outcome but not ETV6/RUNX1. The EFS and OS of the ETV6/RUNX1-positive group were significantly higher than those of the negative group (3-year EFS: 90.11 ± 4.21% vs 82 ± 2.36%, P<0.0001, 3-year OS: 91.99 ± 3.92% vs 88.79 ± 1.87%, P=0.017). Subgroup analysis showed that chemotherapy protocol, age, prednisone response, and D15 MRD were important factors affecting the prognosis of ETV6/RUNX1-positive children.ConclusionsETV6/RUNX1-positive pediatric ALL showed an excellent outcome but lack of independent prognostic significance in South China. However, for older patients who have the ETV6/RUNX1 fusion and slow response to therapy, to opt for more intensive treatment.
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