| Autor: |
Zhao Y; Department of Pediatrics, Peking University People's Hospital, Beijing 100044, China., Huang S; Medical Oncology Department, Pediatric Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing Key Laboratory of Pediatric Hematology Oncology, Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing 100045, China., Jia YP; Department of Pediatrics, Peking University People's Hospital, Beijing 100044, China., Zhang LP; Department of Pediatrics, Peking University People's Hospital, Beijing 100044, China., Duan YL; Medical Oncology Department, Pediatric Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing Key Laboratory of Pediatric Hematology Oncology, Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing 100045, China., Jin L; Medical Oncology Department, Pediatric Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing Key Laboratory of Pediatric Hematology Oncology, Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing 100045, China., Zhai XW; Department of Hematology, Children's Hospital of Fudan University, National Children's Medical Center, Shanghai 201102, China., Wang HS; Department of Hematology, Children's Hospital of Fudan University, National Children's Medical Center, Shanghai 201102, China., Hu B; Department of Pediatric Lymphoma, Beijing GoBroad Boren Hospital, Beijing 100070, China., Liu Y; Department of Pediatric Lymphoma, Beijing GoBroad Boren Hospital, Beijing 100070, China., Liu AS; Department of Hematology & Oncology, Xi'an Children's Hospital, Xi'an 710002, China., Liu W; Department of Hematology & Oncology, Zhengzhou Children's Hospital, Zhengzhou 450018, China., Zheng MC; Department of Hematology, Hunan Children's Hospital, Changsha 410007, China., Li F; Hematology & Oncology Department, Children's Hospital Affiliated to Shandong University, Jinan 250022, China., Sun LR; Department of Pediatric Hematology & Oncology, the Affiliated Hospital of Qingdao University, Qingdao 266003, China., Yuan XJ; Department of Pediatric Hematology/Oncology, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200092, China., Dai YP; Department of Pediatric Hematology & Endocrinology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan 250021, China., Zhang BX; Department of Pediatrics, Second Hospital of Hebei Medical University, Shijiazhuang 050004, China., Jiang L; Department of Pediatrics, Forth Hospital of Hebei Medical University, Shijiazhuang 050004, China., Wang XG; Department of Hematology and Oncology, Third Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China., Wang HM; Department of Pediatric Hematology & Endocrinology, First Hospital of Shandong First Medical University, Jinan 250021, China., Zhou CJ; Pathology Department, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China., Gao ZF; Department of Pathology, Peking University Third Hospital, Beijing 100191, China;Zhao Yang and Huang Shuang contributed equally to the artical., Zhang YH; Department of Pediatric Lymphoma, Beijing GoBroad Boren Hospital, Beijing 100070, China. |
| Abstrakt: |
Objective: To analyze the mid-term efficacy of the China Net Childhood Lymphoma mature B-cell lymphoma 2017 (CNCL-B-NHL-2017) regimen in treating children with high-grade B-cell lymphoma (HGBL). Methods: Clinical and pathological data of HGBL children aged≤18 years admitted to 16 hospitals of the Chinese Children's Lymphoma Collaborative Group (CNCL) from May 2017 to April 2021 were collected retrospectively. They were divided in to high-grade B-cell lymphoma with double hit/triple hit (HGBL-DH/TH) group and high-grade B-cell lymphoma non-specified (HGBL-NOS) group, according to the 2016 version of the World Health Organization (WHO) Hematopoietic and Lymphoid Tissues Cancer Classification. Both groups of patients were treated with stratified chemotherapy by risk according to the CNCL-B-NHL-2017 scheme. The deadline for follow-up was December 31, 2023. All the patients were examined by chromosome fluorescence in situ hybridization (FISH), and the rearrangement of genes MYC, BCL-2 and BCL-6 was confirmed. The clinical and pathological characteristics of patients at disease onset were analyzed, and the therapeutic effects of patients in different clinical stages and risk groups were compared. Survival analysis was drawn by Kaplan Meier method, the log-rank test was used to compare the differences in the cumulative survival rate between different groups, and multivariate Cox regression model was used to identify the prognostic factors. Results: A total of 62 patients were included, with an onset age [ M ( Q 1 , Q 3 )] of 7 (4, 11) years, including 48 males and 14 females. There were 11 (17.7%) patients in stageⅡ, 33(53.2%)patients in stage Ⅲ and 18(29.1%)patients in stage Ⅳ. FISH testing showed that 4 cases (6.5%) were HGBL-DH and 3 (4.8%) were HGBL-TH. The remaining 55 cases (88.7%) were HGBL-NOS, with 18 cases accompanied by MYC rearrangement. There were 7 cases in the HGBL-DH/TH group and 55 cases in the HGBL-NOS group. Thirteen cases (20.9%) were treated with the B1 regimen, 3 cases (4.8%) with B2 regimen, 37 cases (59.6%) with C1 regimen, and 9 cases (14.7%) with the C2 regimen. Forty-eight cases (77.4%) received rituximab therapy at the same time. Five cases (8.0%) progressed during treatment. The follow-up time [ M ( Q 1 , Q 3 )] was 43.5 (36.1, 53.7) months. The complete remission rate was 91.9% (57/62). The 3 year overall survival rate was 93.5% and event-free survival (EFS) rate was 91.9%. The 3-year overall survival rate in the HGBL-NOS group was higher than that in the HGBL-DH/TH group (96.3% vs 71.4%, P =0.011). The 3-year EFS rate of the HGBL-NOS group was higher than that of the HGBL-DH/TH group (94.5% vs 71.4%, P =0.037). In the HGBL-NOS subgroup, the overall survival rate of children with MYC rearrangement was lower (100% vs 88.9%, P =0.039). Multivariate Cox regression analysis showed that central invasion ( HR =6.05, 95% CI : 1.96-38.13, P =0.046) was a risk factor for overall survival. Conclusion: CNCL-B-NHL-2017 regimen shows significant effects in the treatment of pediatric HGBL, with a good prognosis. |
