Clinical features and prognosis of core binding factor acute myeloid leukemia in children.
- Author:
Chao LIU
1
;
Xiao-Yan CHEN
;
Mei-Hui YI
;
Wen-Qi WU
;
Min RUAN
;
Xiao-Fan ZHU
Author Information
1. State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China. xfzhu@ihcams.ac.cn.
- Publication Type:Journal Article
- MeSH:
Child;
Core Binding Factor Alpha 2 Subunit;
Core Binding Factors;
Humans;
Leukemia, Myeloid, Acute;
Oncogene Proteins, Fusion;
Prognosis;
RUNX1 Translocation Partner 1 Protein;
Retrospective Studies
- From:
Chinese Journal of Contemporary Pediatrics
2020;22(7):739-743
- CountryChina
- Language:Chinese
-
Abstract:
OBJECTIVE:To study the clinical features and prognosis of core binding factor acute myeloid leukemia (CBF-AML) in children.
METHODS:A retrospective analysis was performed from the chart review data of children who were newly diagnosed with CBF-AML in the Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences, from August 2009 to November 2015. According to the type of fusion gene, the children were divided into CBFB-MYH11 and AML1-ETO groups. Clinical features and prognosis were analyzed and compared between the two groups.
RESULTS:A total of 91 children with CBF-AML were enrolled in this study, among whom there were 74 (81%) in the AML1-ETO group and 17 (19%) in the CBFB-MYH11 group. Additional chromosomal abnormalities were observed in 38 children (42%), and deletion of sex chromosome was the most common abnormality and was observed in 28 children (31%). After the first course of induction treatment, the complete remission rate was 97% (88/91), the recurrence rate was 29% (26/91), the 5-year event-free survival (EFS) rate was 65%±6%, and the 5-year overall survival (OS) rate was 75%±5%. There were no significant differences between the AML1-ETO and CBFB-MYH11 groups in 5-year EFS rate (62%±7% vs 77%±11%, P>0.05) or 5-year OS rate (72%±6% vs 88%±9%, P>0.05).
CONCLUSIONS:AML1-ETO is the main type of fusion gene in children with CBF-AML, and deletion of sex chromosome is the most common type of additional chromosomal abnormalities. Children with CBF-AML often have a good prognosis, and the children with AML1-ETO have a similar prognosis to those with CBFB-MYH11.
