Clinicopathological analysis of 3 pediatric cases of Epstein-Barr virus-positive dif-fuse large B-cell lymphoma
10.13315/j.cnki.cjcep.2025.08.008
- VernacularTitle:儿童EB病毒阳性弥漫性大B细胞淋巴瘤3例临床病理学分析
- Author:
Hanxiang SONG
1
;
Mianfu CAO
;
Qiulin TAN
;
Bo LIU
;
Gang MENG
Author Information
1. 陆军军医大学第二附属医院病理科,重庆 400037
- Publication Type:Journal Article
- Keywords:
lymphoma;
diffuse large B-cell lymphoma;
pediatric;
Epstein-Barr virus;
gene rearrangement
- From:
Chinese Journal of Clinical and Experimental Pathology
2025;41(8):1024-1030
- CountryChina
- Language:Chinese
-
Abstract:
Purpose To investigate the clinicopathological features and molecular genetic alteration of Epstein-Barr virus-positive diffuse large B-cell lymphoma(EBV+DLBCL)in pediatric patients.Methods Clinical data were collected for 3 pediatric patients diagnosed with EBV+DLBCL.Immunohistochemistry(EnVision two-step method)was used to evaluate expression of CD20,CD79α,CD3,CD5,CD30,EBNA2,Fascin,and GATA3.In situ hybridization detected EBER1/2 expression.B-cell receptor(BCR)and T-cell receptor(TCR)gene rearrangements were performed to evaluate the clonal rearrangement of tumor lymphocytes.Results Among the 3 pediatric patients(ages 13-18),there were 2 males and 1 female.All patients presented with painless lymphadenopathy without bone marrow involve-ment.One female patient exhibited B symptoms(fever,night sweats,and weight loss),whereas both male patients were asymptomatic.According to the Lugano classification,2 cases were stage Ⅲand one was stage Ⅳ.Histopathologi-cally,2 cases exhibited a polymorphic morphology resembling T-cell/histiocyte-rich large B-cell lymphoma,and one case demonstrated monomorphic morphology typical of conventional DLBCL.Immunophenotypically,all cases strongly expressed various B-cell transcription factors;CD30 expression varied in intensity;EBNA2,Fascin and GATA3 were uniformly negative.In situ hybridization indicated EBER1/2 positive expression in large tumor cells and scattered back-ground small lymphocytes.Clonal Ig gene rearrangement peaks were detected in all 3 cases.Each patient received standard DLBCL chemotherapy,and follow-up PET-CT scans indicated complete remission in all.Conclusion Pediat-ric EBV+DLBCL is rare,and diagnosing the polymorphic subtype poses particular challenges.In China,many patholo-gists consider detection of clonal Ig gene rearrangement as the diagnostic"gold standard".Even when clinical course and imaging data strongly support EBV+DLBCL,final diagnosis can remain controversial.Further accumulation of ca-ses and long-term follow-up are needed to elucidate optimal diagnostic criteria,treatment responses,and prognostic fac-tors.
