Clinical and pathological characteristics and prognostic analysis of large B-cell lymphoma with IRF4 rearrangement
10.13315/j.cnki.cjcep.2024.07.013
- VernacularTitle:伴IRF4重排的大B细胞淋巴瘤的临床病理特征及预后分析
- Author:
Shi HE
1
;
Chengyu WU
;
Shuyi LU
;
Longfeng KE
;
Yating QIU
;
Wenwen ZHANG
;
Gang CHEN
;
Yanping CHEN
Author Information
1. 福建医科大学肿瘤临床医学院/福建省肿瘤医院病理科,福州 350014
- Keywords:
large B-cell lymphoma;
IRF4;
rearrangement;
MUM-1;
immunohistochemistry;
diagnosis
- From:
Chinese Journal of Clinical and Experimental Pathology
2024;40(7):730-735
- CountryChina
- Language:Chinese
-
Abstract:
Purpose To explore the clinicopathological features,diagnosis and differential diagnosis of large B-cell lym-phoma with IRF4 rearrangement(LBCL-IRF4r).Methods Clinical data of 8 cases of LBCL-IRF4r were collect,hematoxy-lin-eosin and immunohistochemical of EnVision two-step stains,in situ hybridization and FISH was used to study the histology,immunotypes and molecular genetic characteristics.The rele-vant literatures were reviewed.Results Among 8 cases of LBCL-IRF4r,the male to female ratio was 1.67:1,with age range 10-53 years(mean 25.8 years).Five cases occurred in tonsils,2 cases in nasopharynx and 1 cases in inguinal lymph node.Microscopically,the tumors presented with a purely follic-ular,purely diffuse or a combined follicular and diffuse architec-ture.The tumor cells were typical centroblasts and less frequent-ly medium-sized blastic cells with smaller nucleoli,apoptosis and nuclear fragmentation were easily seen.Immunophenotypi-cally,the tumor cells of the eight cases of LBCL-IRF4r diffuse strongly expressed CD20(8/8),PAX5(2/2),CD79a(3/3),BCL6(8/8)and MUM-1(8/8),mostly expressed CD10(7/8),partially expressed BCL2(5/8)and CD5(4/8),and did not express Cyclin D1,CD23 and CD30.The percentage of Ki67 index ranged from 70%to 95%.EBER in situ hybridiza-tion was negative in all cases.IRF4 rearrangements were detec-ted in all cases(8/8).BCL6 rearrangements were detected in one case(1/2).MYC(0/4)and BCL2(0/3)rearrangements were not detected in all cases.Conclusion LBCL-IRF4r is more common in children and adolescents with characteristic IG::IRF4 rearrangement and a good prognosis,which needs to be differentiated from other types of large B-cell lymphoma.