Blood Res 2022; 57(1):
Published online March 31, 2022
https://doi.org/10.5045/br.2021.2021164
© The Korean Society of Hematology
Correspondence to : Hyery Kim, M.D., Ph.D.
Division of Pediatric Hematology/Oncology, Department of Pediatrics, University of Ulsan College of Medicine, Asan Medical Center, 88-1 Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea
E-mail: taban@hanmail.net
*This study was supported by a National Research Foundation of Korea grant, funded by the Korean government(Ministry of Science and ICT) (no. NRF-2018R1C1B5047092).
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Background
Aggressive mature B-cell non-Hodgkin lymphoma (B-NHL) is the most common non-Hodgkin lymphoma in children. The outcome of chemotherapy for B-NHL has improved over decades.
Methods
We reviewed 82 children and adolescents with B-NHL diagnosed at Asan Medical Center between 1993 and 2020. The D-COMP/COMP (daunomycin–cyclophosphamide, doxorubicin, vincristine, and prednisolone), Pediatric Oncology Group (POG)-9219/9315/9317, R-CHOP/CHOP (rituximab–cyclophosphamide, doxorubicin, vincristine, and prednisolone), and Lymphomes Malins B 89 (LMB89)/LMB96 regimens were administered. In 2018, rituximab was added to the LMB protocol (R-LMB) for advanced-staged Burkitt lymphoma (BL). The patients’ clinical features and treatment outcomes were retrospectively analyzed.
Results
The most common subtype was BL (61%), followed by diffuse large B-cell lymphoma (DLBCL) (35%). The median age was 7.8 (range, 1.3‒16.4) years, and the most frequently used regimen was French‒American‒British (FAB)/LMB96 (58 patients, 70.7%). The 5-year overall survival (OS) and event-free survival (EFS) rates were 92.5% and 85.7%, respectively. The EFS rates of patients with BL and DLBCL were 90.0% and 79.3%, respectively. Among the FAB/LMB risk groups, group C (85.7%) had a significantly lower 5-year OS (P =0.037). Eleven events occurred (6 relapses, 3 deaths, and 2 secondary malignancies) during the median follow-up of 7.1 (range, 3.7‒118.5) months. Two patients treated with R-LMB had good outcomes without complications.
Conclusion
Various treatment regimens have favorable outcomes in pediatric patients with B-NHL. However, further studies are needed to improve survival in high-risk patients. In addition, careful monitoring for acute toxicity or secondary malignancy due to intensive multidrug chemotherapy is required.
Keywords Mature B-cell lymphoma, Children, Survival, LMB protocol, Rituximab
Blood Res 2022; 57(1): 41-50
Published online March 31, 2022 https://doi.org/10.5045/br.2021.2021164
Copyright © The Korean Society of Hematology.
Woojung Jeon1, Young Kwon Koh1,2, Sunghan Kang1,2, Hyery Kim1,2, Kyung-Nam Koh1,2, Ho Joon Im1,2
1Department of Pediatrics, University of Ulsan College of Medicine, Asan Medical Center, 2Division of Pediatric Hematology/Oncology, Department of Pediatrics, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea
Correspondence to:Hyery Kim, M.D., Ph.D.
Division of Pediatric Hematology/Oncology, Department of Pediatrics, University of Ulsan College of Medicine, Asan Medical Center, 88-1 Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea
E-mail: taban@hanmail.net
*This study was supported by a National Research Foundation of Korea grant, funded by the Korean government(Ministry of Science and ICT) (no. NRF-2018R1C1B5047092).
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Background
Aggressive mature B-cell non-Hodgkin lymphoma (B-NHL) is the most common non-Hodgkin lymphoma in children. The outcome of chemotherapy for B-NHL has improved over decades.
Methods
We reviewed 82 children and adolescents with B-NHL diagnosed at Asan Medical Center between 1993 and 2020. The D-COMP/COMP (daunomycin–cyclophosphamide, doxorubicin, vincristine, and prednisolone), Pediatric Oncology Group (POG)-9219/9315/9317, R-CHOP/CHOP (rituximab–cyclophosphamide, doxorubicin, vincristine, and prednisolone), and Lymphomes Malins B 89 (LMB89)/LMB96 regimens were administered. In 2018, rituximab was added to the LMB protocol (R-LMB) for advanced-staged Burkitt lymphoma (BL). The patients’ clinical features and treatment outcomes were retrospectively analyzed.
Results
The most common subtype was BL (61%), followed by diffuse large B-cell lymphoma (DLBCL) (35%). The median age was 7.8 (range, 1.3‒16.4) years, and the most frequently used regimen was French‒American‒British (FAB)/LMB96 (58 patients, 70.7%). The 5-year overall survival (OS) and event-free survival (EFS) rates were 92.5% and 85.7%, respectively. The EFS rates of patients with BL and DLBCL were 90.0% and 79.3%, respectively. Among the FAB/LMB risk groups, group C (85.7%) had a significantly lower 5-year OS (P =0.037). Eleven events occurred (6 relapses, 3 deaths, and 2 secondary malignancies) during the median follow-up of 7.1 (range, 3.7‒118.5) months. Two patients treated with R-LMB had good outcomes without complications.
Conclusion
Various treatment regimens have favorable outcomes in pediatric patients with B-NHL. However, further studies are needed to improve survival in high-risk patients. In addition, careful monitoring for acute toxicity or secondary malignancy due to intensive multidrug chemotherapy is required.
Keywords: Mature B-cell lymphoma, Children, Survival, LMB protocol, Rituximab
Table 1 . Characteristics of the patients..
Characteristics | N=82 (%) |
---|---|
Age at diagnosis, median (range, yr) | 7.8 (1.3–16.4) |
Sex | |
Male | 65 (79) |
Female | 17 (21) |
Primary site | |
Abdomen | 43 (52.4) |
Head and neck, except skin and nodes | 17 (20.7) |
Peripheral lymph nodes | 11 (13.4) |
Thorax | 5 (6.1) |
Others | 4 (5) |
Central nervous system | 2 (2.4) |
Pathologic subtype | |
Burkitt lymphoma | 50 (61.0) |
Diffuse large B-cell lymphoma | 29 (35.4) |
High-grade B-cell lymphoma, not otherwise specified | 3 (3.6) |
BM involvement | |
Yes | 14 (17) |
No | 68 (83) |
CNS involvement | |
Yes | 8 (9.8) |
No | 74 (90.2) |
Stage | |
I | 4 (4.9) |
II | 26 (31.7) |
III | 30 (36.6) |
IV | 22 (26.8) |
Chemotherapy protocol and group | |
LMB89/LMB96 | 68 (82.9) |
Group A | 6 (8.8) |
Group B | 35 (51.5) |
Group C | 27 (39.7) |
POG-9219/9315/9317 | 9 (11.0) |
D-COMP/COMP | 3 (3.7) |
R-CHOP/CHOP | 2 (2.4) |
Abbreviations: BM, bone marrow; CNS, central nervous system..
Table 2 . Characteristics of the events..
Event characteristics | |
---|---|
Total events (N of patients) | 11 |
Relapse (N) | 6 |
Local relapse | 4 |
BM relapse | 1 |
CNS relapse | 1 |
Median time to relapse (range, mo) | 6.8 (4.3–13.9) |
Pathologic subtype | |
Burkitt lymphoma | 2 (33.3%) |
Diffuse large B-cell lymphoma | 4 (66.7%) |
Stage | |
II | 2 (33.3%) |
III | 3 (50.0%) |
IV | 1 (16.7%) |
Salvage regimen | |
CTx only | 4 (66.7%) |
CTx+RTx | 1 (16.7%) |
CTx+RTx+allo-HSCT | 1 (16.7%) |
Death after relapse (N) | 4 |
Death of any cause other than relapse (N) | 3 |
Progressive disease | 2 |
Treatment related | 1 |
Median time to death (range, mo) | 10.5 (3.7–27.1) |
Secondary malignancy (N) | 2 |
Therapy-related acute myeloid leukemia | 1 |
Follicular thyroid carcinoma | 1 |
Abbreviations: BM, bone marrow; CNS, central nervous system; CTx, chemotherapy; HSCT, hematopoietic stem cell transplantation; RTx, radiotherapy..
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