Non-germinal center B-cell subtype of pediatric diffuse large B-cell lymphoma in Japan: A retrospective cohort study

Daiki Hori; Ryoji Kobayashi; Atsuko Nakazawa; Hideto Iwafuchi; Wolfram Klapper; Tomoo Osumi; Kentaro Ohk; Masahiro Sekimizu

doi:10.1002/pbc.30279

Non-germinal center B-cell subtype of pediatric diffuse large B-cell lymphoma in Japan: A retrospective cohort study

Pediatr Blood Cancer. 2023 May;70(5):e30279. doi: 10.1002/pbc.30279. Epub 2023 Mar 1.

Authors

Daiki Hori^{1

2}, Ryoji Kobayashi^{1

2}, Atsuko Nakazawa^{1

3}, Hideto Iwafuchi^{1

4}, Wolfram Klapper⁵, Tomoo Osumi⁶, Kentaro Ohk^{1

7}, Masahiro Sekimizu^{1

8}

Affiliations

¹ Lymphoma and Pathology Committee, JCCG (Japan Children's Cancer Group)/JPLSG (Japan Pediatric Leukemia/Lymphoma Study Group), Tokyo, Japan.
² Department of Hematology/Oncology for Children and Adolescents, Sapporo Hokuyu Hospital, Sapporo, Hokkaido, Japan.
³ Department of Clinical Research, Saitama Children's Medical Center, Saitama, Japan.
⁴ Department of Pathology, Shizuoka Children's Hospital, Shizuoka, Japan.
⁵ Department of Pathology, General Pathology and Hematopathology, University Hospitals Schleswig-Holstein, Kiel, Germany.
⁶ National Center for Child Health and Development, Children's Cancer Center, Tokyo, Japan.
⁷ Department of Pediatric Hematology and Oncology Research, Research Institute, National Center for Child Health and Development, Tokyo, Japan.
⁸ Department of Pediatrics, National Hospital Organization Nagoya Medical Center, Nagoya, Japan.

PMID: 36860130
DOI: 10.1002/pbc.30279

Abstract

Background: Diffuse large B-cell lymphoma (DLBCL) is classified into two molecular subtypes according to its cell of origin: germinal center B-cell (GCB) subtype and activated B-cell/non-GCB subtype. This latter subtype shows a poorer prognosis in adults. However, in pediatric DLBCL, the prognostic impact of the subtype is yet to be clarified.

Objectives: This study sought to compare the prognosis between GCB and non-GCB DLBCL in a large number of cases in children and adolescents. In addition, this study intended to describe the clinical, immunohistochemical, and cytogenetic characteristics of these two molecular subtypes of DLBCL, and consider differences in the biology, frequency, and prognosis of GCB and non-GCB subtypes in pediatric versus adult DLBCL or in Japanese versus Western pediatric DLBCL patients.

Design/methods: We selected mature B-cell lymphoma/leukemia patients for whom specimens had been submitted to the central pathology review in Japan between June 2005 and November 2019. We referred the past studies on Asian adult patients and Western pediatric patients to compare with our results.

Results: Data were obtained from 199 DLBCL patients. The median age of all patients was 10 years, with 125 patients (62.8%) in the GCB group and 49 (24.6%) in the non-GCB group other than 25 cases whose immunohistochemical data were insufficient. Overall, the percentage of translocation of MYC (1.4%) and BCL6 (6.3%) was lower than in adult and Western pediatric DLBCL cases. The non-GCB group showed a significantly higher proportion of females (44.9%), a higher incidence of stage III disease (38.8%), and B-cell lymphoma 2 (BCL2)-positivity in immunohistochemistry (79.6%) compared to the GCB group; however, no BCL2 rearrangement was observed in both GCB and non-GCB groups. The prognosis did not differ significantly between the GCB and non-GCB groups.

Conclusion: This study including a large number of non-GCB patients showed the same prognosis between GCB and non-GCB groups and suggested a difference in the biology of pediatric and adolescent DLBCL compared to adult DLBCL as well as between Asian and Western DLBCL.

Keywords: adolescent; diffuse large B-cell lymphoma; germinal center B-cell-like; non-germinal center B-cell-like; pediatric.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adolescent
Adult
B-Lymphocytes
Child
Female
Humans
Japan / epidemiology
Lymphoma, Large B-Cell, Diffuse* / epidemiology
Lymphoma, Large B-Cell, Diffuse* / pathology
Prognosis
Retrospective Studies