Effect of increasing the intensity of chemotherapy on the prognosis of acute lymphoblastic leukemia in children with IKZF1 deletion.
- Author:
Yong-Zhi ZHENG
1
;
Jian LI
;
Shao-Hua LE
;
Hao ZHENG
;
Xue-Ling HUA
;
Zai-Sheng CHEN
;
Jian-Da HU
Author Information
1. Department of Pediatric Hematology, Fujian Institute of Hematology, Fujian Provincial Key Laboratory, Fujian Medical University Union Hospital, Fuzhou 350001, China. 1354113723@qq.com.
- Publication Type:Journal Article
- MeSH:
Disease-Free Survival;
Gene Deletion;
Humans;
Ikaros Transcription Factor;
genetics;
Neoplasm, Residual;
Precursor Cell Lymphoblastic Leukemia-Lymphoma;
Prognosis
- From:
Chinese Journal of Contemporary Pediatrics
2019;21(7):690-695
- CountryChina
- Language:Chinese
-
Abstract:
OBJECTIVE:To study the clinical features of acute lymphoblastic leukemia (ALL) in children with IKAROS family zinc finger 1 (IKZF1) deletion, and to observe the effect of increasing the intensity of chemotherapy on the prognosis of this disease.
METHODS:A total of 278 children diagnosed with ALL between December 2015 and February 2018 were systematically treated according to the Chinese Children's Leukemia Group-ALL 2008 protocol (CCLG-ALL 2008). The patients were divided into an IKZF1-deleted group and a control group according to the presence or absence of IKZF1. The IKZF1-deleted group was treated with the regimen for high-risk group (HR) in the CCLG-ALL 2008 protocol, while the control group received different intensities of chemotherapy according to clinical risk classification. The clinical features and event-free survival rate (EFS) were compared between the two groups.
RESULTS:A total of 24 (8.6%) cases of 278 children were found to have large deletions of exons of the IKZF1 gene. The IKZF1-deleted group had significantly higher proportions of cases with white blood cell count ≥50×10/L at initial diagnosis, BCR-ABL1 fusion gene positive, minimal residual disease ≥10% on the 15th day of induction remission treatment, minimal residual disease-high risk and clinical risk classification-high risk compared with the control group (P<0.05). The 3-year EFS rate (76%±10%) in the IKZF1-deleted group was lower than that in the control group (84%±4%), but with no significant difference between the two groups (P=0.282). The estimated 3-year EFS rate in the IKZF1-deleted-non-HR group (actually treated with the chemotherapy regimen for HR in the CCLG-ALL 2008 protocol) was 82%±12%, which was lower than that in the control-non-HR group (86%±5%), but there was no significant difference (P=0.436).
CONCLUSIONS:ALL children with IKZF1 deletion have worse early treatment response, and increasing the intensity of chemotherapy might improve the prognosis.
