Mid-term efficacy of China Net Childhood Lymphoma-mature B-cell lymphoma 2017 regimen in the treatment of pediatric Burkitt lymphoma.
- Author:
Meng ZHANG
1
;
Pan WU
2
;
Yan Long DUAN
1
;
Ling JIN
1
;
Jing YANG
1
;
Shuang HUANG
1
;
Ying LIU
3
;
Bo HU
3
;
Xiao Wen ZHAI
4
;
Hong Sheng WANG
4
;
Yang FU
4
;
Fu LI
5
;
Xiao Mei YANG
5
;
An Sheng LIU
6
;
Shuang QIN
6
;
Xiao Jun YUAN
7
;
Yu Shuang DONG
7
;
Wei LIU
8
;
Jian Wen ZHOU
8
;
Le Ping ZHANG
9
;
Yue Ping JIA
9
;
Jian WANG
10
;
Li Jun QU
10
;
Yun Peng DAI
11
;
Guo Tao GUAN
11
;
Li Rong SUN
12
;
Jian JIANG
12
;
Rong LIU
13
;
Run Ming JIN
14
;
Zhu Jun WANG
14
;
Xi Ge WANG
15
;
Bao Xi ZHANG
16
;
Kai Lan CHEN
17
;
Shu Quan ZHUANG
18
;
Jing ZHANG
19
;
Chun Ju ZHOU
20
;
Zi Fen GAO
21
;
Min Cui ZHENG
2
;
Yonghong ZHANG
1
Author Information
- Publication Type:Journal Article
- MeSH: Adolescent; Antineoplastic Combined Chemotherapy Protocols/therapeutic use*; Burkitt Lymphoma/drug therapy*; Child; Disease-Free Survival; Female; Humans; Lactate Dehydrogenases; Lymphoma, B-Cell/drug therapy*; Male; Prognosis; Retrospective Studies; Rituximab/therapeutic use*; Treatment Outcome
- From: Chinese Journal of Pediatrics 2022;60(10):1011-1018
- CountryChina
- Language:Chinese
- Abstract: Objective: To analyze the clinical characteristics of children with Burkitt lymphoma (BL) and to summarize the mid-term efficacy of China Net Childhood Lymphoma-mature B-cell lymphoma 2017 (CNCL-B-NHL-2017) regimen. Methods: Clinical features of 436 BL patients who were ≤18 years old and treated with the CNCL-B-NHL-2017 regimen from May 2017 to April 2021 were analyzed retrospectively. Clinical characteristics of patients at disease onset were analyzed and the therapeutic effects of patients with different clinical stages and risk groups were compared. Survival analysis was performed by Kaplan-Meier method, and Cox regression was used to identify the prognostic factors. Results: Among 436 patients, there were 368 (84.4%) males and 68 (15.6%) females, the age of disease onset was 6.0 (4.0, 9.0) years old. According to the St. Jude staging system, there were 4 patients (0.9%) with stage Ⅰ, 30 patients (6.9%) with stage Ⅱ, 217 patients (49.8%) with stage Ⅲ, and 185 patients (42.4%) with stage Ⅳ. All patients were stratified into following risk groups: group A (n=1, 0.2%), group B1 (n=46, 10.6%), group B2 (n=19, 4.4%), group C1 (n=285, 65.4%), group C2 (n=85, 19.5%). Sixty-three patients (14.4%) were treated with chemotherapy only and 373 patients (85.6%) were treated with chemotherapy combined with rituximab. Twenty-one patients (4.8%) suffered from progressive disease, 3 patients (0.7%) relapsed, and 13 patients (3.0%) died of treatment-related complications. The follow-up time of all patients was 24.0 (13.0, 35.0) months, the 2-year event free survival (EFS) rate of all patients was (90.9±1.4) %. The 2-year EFS rates of group A, B1, B2, C1 and C2 were 100.0%, 100.0%, (94.7±5.1) %, (90.7±1.7) % and (85.9±4.0) %, respectively. The 2-year EFS rates was higher in group A, B1, and B2 than those in group C1 (χ2=4.16, P=0.041) and group C2 (χ2=7.21, P=0.007). The 2-year EFS rates of the patients treated with chemotherapy alone and those treated with chemotherapy combined with rituximab were (79.3±5.1)% and (92.9±1.4)% (χ2=14.23, P<0.001) respectively. Multivariate analysis showed that stage Ⅳ (including leukemia stage), serum lactate dehydrogenase (LDH)>4-fold normal value, and with residual tumor in the mid-term evaluation were risk factors for poor prognosis (HR=1.38,1.23,8.52,95%CI 1.05-1.82,1.05-1.43,3.96-18.30). Conclusions: The CNCL-B-NHL-2017 regimen show significant effect in the treatment of pediatric BL. The combination of rituximab improve the efficacy further.
