Clinical characteristics and prognosis analysis of childhood B-cell acute lymphoblastic leukemia with TEL-AML1 fusion gene positive
10.3760/cma.j.issn.1009-9921.2019.10.003
- VernacularTitle: TEL-AML1融合基因阳性儿童急性B淋巴细胞白血病临床特点及预后分析
- Author:
Hui KANG
1
;
Guoping HAO
2
;
Xiaohuan WANG
2
;
Yanli CHENG
2
Author Information
1. Department of Pediatrics, Shanxi Medical University, Taiyuan 030001, China
2. Department of Hematology, Children's Hospital of Shanxi, Taiyuan 030013, China
- Publication Type:Journal Article
- Keywords:
Leukemia, B-cell;
Gene fusion;
Child;
Survival;
Prognosis
- From:
Journal of Leukemia & Lymphoma
2019;28(10):588-592
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinical characteristics and prognosis of children B-cell acute lymphoblastic leukemia (B-ALL) with TEL-AML1 fusion gene positive.
Methods:Clinical characteristics, therapeutic effects and prognostic factors of 55 children B-ALL patients with TEL-AML1 fusion gene positive in Children's Hospital of Shanxi from January 2013 to June 2018 were retrospectively analyzed. Kaplan-Meier method was used to evaluate 3-year event-free survival (EFS) rate and overall survival (OS) rate. Influencing factors of EFS and OS were evaluated by using Cox regression analysis.
Results:TEL-AML1 fusion gene was positive in all 55 children, and no other fusion gene positive was merged. There were 4 patients (7.3%) ≥10 years old. At initial diagnosis, 33 patients (60.0%) had hepatomegaly, 28 patients (50.9%) had splenomegaly, and 27 patients (49.1%) had superficial lymphadenectasis. There were 5 patients (9.1%) with white blood cell count ≥50×109/L, and 19 patients (34.6%) had abnormalities of chromosome. All the 55 children were divided into the low risk group [36 cases (65.5%)], the intermediate risk group [18 cases(32.7%)], high risk group [1 case (1.8%)] according to Morphology, Immunology, Cytogenetics and Molecular Biology (MICM) and adjusted risk. After regular treatments, 50 patients achieved complete remission (CR) on the 15th day. The CR rate after one-course of induction therapy was 100.0%. On the 33rd day, 43 patients (78.2%) had minimal residual disease (MRD) <10-4, 12 patients (21.8%) had MRD≥10-4 and MRD<10-2, 1 patient (1.8%) had MRD≥10-3 at the 12th week. During three to six months, the negative rate of fusion gene was 61.8% (34/55). There were 3 deaths (5.5%), and one (1.8%) of them died of recurrence, and the recurrence time was 27 months from the initial diagnosis; the other 2 cases (3.6%) died of infection during chemotherapy. In 55 patients, the 3-year EFS rate and OS rate was 90.3% and 93.2%, respectively. The 3-year EFS rate and OS rate in the low risk group was 100.0% both; the 3-year EFS rate and OS rate in the intermediate risk group was 78.7% and 86.6%, respectively; the 3-year EFS rate and OS rate in the high risk group was 0 both and one died. EFS rate and OS rate in low risk group were higher than those in the intermediate risk group, and the differences were statistically significant (P < 0.05). The EFS rate was 92.0% and 0 at the 12th week MRD<10-3 group and MRD≥10-3 group, and OS rate was 95.0% and 0 at the 12th week MRD<10-3 group and MRD≥10-3 group (P < 0.05). Cox multivariate analysis showed that MRD at the 12th week was an independent risk factor influencing EFS and OS (OR= 2.971, 95% CI 1.330-6.633, P= 0.008; OR= 2.884, 95% CI 1.295-6.419, P= 0.009).
Conclusions:Children B-ALL patients with TEL-AML1 fusion gene positive have a low recurrence rate, high survival rate and good prognosis. Risk stratification and the 12th week MRD are the influencing factors of the prognosis.
