Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2022 Mar 31;57(1):41-50.
doi: 10.5045/br.2021.2021164.

Clinical characteristics and treatment outcomes of children and adolescents with aggressive mature B-cell lymphoma: a single-center analysis

Woojung Jeon  1 , Young Kwon Koh  1   2 , Sunghan Kang  1   2 , Hyery Kim  1   2 , Kyung-Nam Koh  1   2 , Ho Joon Im  1   2
Affiliations

Clinical characteristics and treatment outcomes of children and adolescents with aggressive mature B-cell lymphoma: a single-center analysis

Woojung Jeon et al. Blood Res. .

Abstract

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: Children; LMB protocol; Mature B-cell lymphoma; Rituximab; Survival.

PubMed Disclaimer

Conflict of interest statement

Authors’ Disclosures of Potential Conflicts of Interest

No potential conflicts of interest relevant to this article were reported.

Figures

Fig. 1
Fig. 1
Events and outcomes according to treatment protocol. Abbreviations: HLH, hemophagocytic lymphohistiocytosis; HSCT, hematopoietic stem cell transplantation; NOS, not otherwise specified; RTx, radiotherapy; t-AML, therapy-related acute myeloid leukemia.
Fig. 2
Fig. 2
Survival outcomes of the 82 patients.
Fig. 3
Fig. 3
Treatment outcomes according to histopathologic subtype: overall survival (A) and event-free survival (B) rates. Abbreviation: NOS, not otherwise specified.
Fig. 4
Fig. 4
Overall survival (A) and event-free survival (B) rates of patients with Burkitt lymphoma and diffuse large B-cell lymphoma according to initial stage.
Fig. 5
Fig. 5
Survival outcomes of patients with Burkitt lymphoma and diffuse large B-cell lymphoma treated with the LMB protocols according to risk group classification: overall survival (A) and event-free survival (B) rates.
Fig. 6
Fig. 6
Survival outcomes of patients with Burkitt lymphoma and diffuse large B-cell lymphoma according to the initial chemotherapy: overall survival (A) and event-free survival (B) rates.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Davies K, Barth M, Armenian S, et al. Pediatric aggressive mature B-cell lymphomas, version 2.2020, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw. 2020;18:1105–23. doi: 10.6004/jnccn.2020.0036. - DOI - PubMed
    1. Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016;127:2375–90. doi: 10.1182/blood-2016-01-643569. - DOI - PMC - PubMed
    1. Gerrard M, Cairo MS, Weston C, et al. Excellent survival following two courses of COPAD chemotherapy in children and adolescents with resected localized B-cell non-Hodgkin's lymphoma: results of the FAB/LMB 96 international study. Br J Haematol. 2008;141:840–7. doi: 10.1111/j.1365-2141.2008.07144.x. - DOI - PubMed
    1. Cairo MS, Beishuizen A. Childhood, adolescent and young adult non-Hodgkin lymphoma: current perspectives. Br J Haematol. 2019;185:1021–42. doi: 10.1111/bjh.15764. - DOI - PMC - PubMed
    1. Patte C, Auperin A, Michon J, et al. The Société Française d'Oncologie Pédiatrique LMB89 protocol: highly effective multiagent chemotherapy tailored to the tumor burden and initial response in 561 unselected children with B-cell lymphomas and L3 leukemia. Blood. 2001;97:3370–9. doi: 10.1182/blood.V97.11.3370. - DOI - PubMed