Clinical features and prognosis of core binding factor acute myeloid leukemia children in South China: a multicenter study.
10.3760/cma.j.cn112140-20230224-00126
- Author:
Bi Yun GUO
1
;
Yue WANG
1
;
Jian LI
2
;
Chun Fu LI
3
;
Xiao Qin FENG
4
;
Min Cui ZHENG
5
;
Si Xi LIU
6
;
Li Hua YANG
7
;
Hua JIANG
8
;
Hong Gui XU
9
;
Xiang Ling HE
10
;
Hong WEN
1
Author Information
1. Department of Pediatrics, the First Affiliated Hospital of Xiamen University, Xiamen 361003, China.
2. Department of Pediatrics, Fujian Medical University Union Hospital, Fuzhou 350001, China.
3. Nanfang-Chunfu Children's Institute of Hematology & Oncology, Taixin Hospital, Dongguan 523128, China.
4. Department of Pediatrics, Nanfang Hospital, Southern Medical University, Guangzhou 510515, China.
5. Hematology and Oncology, Hunan Children's Hospital, Changsha 410007, China.
6. Department of Hematology, Shenzhen Children's Hospital, Shenzhen 518026, China.
7. Department of Pediatrics, Zhujiang Hospital, Southern Medical University, Guangzhou 510280, China.
8. Hematology and Oncology, Guangzhou Women and Children's Medical Center, Guangzhou 510145, China.
9. Department of Pediatrics, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou 510120, China.
10. Children's Medical Center, People's Hospital of Hunan Province, Changsha 410002, China.
- Publication Type:Journal Article
- MeSH:
Male;
Female;
Humans;
Child;
Retrospective Studies;
RUNX1 Translocation Partner 1 Protein/genetics*;
Core Binding Factor Alpha 2 Subunit/therapeutic use*;
Prognosis;
Leukemia, Myeloid, Acute/genetics*;
Cytarabine/therapeutic use*;
Oncogene Proteins, Fusion/genetics*;
Chromosome Aberrations
- From:
Chinese Journal of Pediatrics
2023;61(10):881-888
- CountryChina
- Language:Chinese
-
Abstract:
Objective: To analyze the clinical features, efficacy and prognosis factors of core binding factor (CBF) acute myeloid leukemia (AML) children in South China. Methods: This was a retrospective cohort study. Clinical data of 584 AML patients from 9 hospitals between January 2015 to December 2020 was collected. According to fusion gene results, all patients were divided into two groups: CBF-AML group (189 cases) and non-CBF-AML group (395 cases). CBF-AML group were divided into AML1-ETO subgroup (154 cases) and CBFβ-MYH11 subgroup (35 cases). Patients in CBF-AML group chosen different induction scheme were divided into group A (fludarabine, cytarabine, granulocyte colony stimulating factor and idarubicin (FLAG-IDA) scheme, 134 cases) and group B (daunorubicin, cytarabine and etoposide (DAE) scheme, 55 cases). Age, gender, response rate, recurrence rate, mortality, molecular genetic characteristics and other clinical data were compared between groups. Kaplan-Meier method was used for survival analysis and survival curve was drawn. Cox regression model was used to analyze prognostic factors. Results: A total of 584 AML children were diagnosed, including 346 males and 238 females. And a total of 189 children with CBF-AML were included, including 117 males and 72 females. The age of diagnosis was 7.3 (4.5,10.0)years, and the white blood cell count at initial diagnosis was 21.4 (9.7, 47.7)×109/L.The complete remission rate of the first course (CR1) of induction therapy, relapse rate, and mortality of children with CBF-AML were significantly different from those in the non-CBF-AML group (91.0% (172/189) vs. 78.0% (308/395); 10.1% (19/189) vs. 18.7% (74/395); 13.2% (25/189) vs. 25.6% (101/395), all P<0.05). In children with CBF-AML, the CBFβ-MYH11 subgroup had higher initial white blood cells and lower proportion of extramedullary invasion than the AML1-ETO subgroup, with statistical significance (65.7% (23/35) vs. 14.9% (23/154), 2.9% (1/35) vs. 16.9% (26/154), both P<0.05). AML1-ETO subgroup had more additional chromosome abnormalities (75/154), especially sex chromosome loss (53/154). Compared with group B, group A had more additional chromosome abnormalities and a higher proportion of tumor reduction regimen, with statistical significance (50.0% (67/134) vs. 29.1% (16/55), 34.3% (46/134) vs. 18.2% (10/55), both P<0.05). Significant differences were found in 5-years event free survival (EFS) rate and 5-year overall survival (OS) rate between CBF-AML group and non-CBF-AML group ((77.0±6.4)%vs. (61.9±6.7)%,(83.7±9.0)%vs. (67.3±7.2)%, both P<0.05).EFS and OS rates of AML1-ETO subgroup and CBFβ-MYH11 subgroup in children with CBF-AML were not significantly different (both P>0.05). Multivariate analysis showed in the AML1-ETO subgroup, CR1 rate and high white blood cell count (≥50×109/L) were independent risk factors for EFS (HR=0.24, 95%CI 0.07-0.85,HR=1.01, 95%CI 1.00-1.02, both P<0.05) and OS (HR=0.24, 95%CI 0.06-0.87; HR=1.01, 95%CI 1.00-1.02; both P<0.05). Conclusions: In CBF-AML, AML1-ETO is more common which has a higher extramedullary involvement and additional chromosome abnormalities, especially sex chromosome loss. The prognosis of AML1-ETO was similar to that of CBFβ-MYH11. The selection of induction regimen group FLAG-IDA for high white blood cell count and additional chromosome abnormality can improve the prognosis.
