Clinicopathological features and prognosis of large B-cell lymphoma with IRF4 re-arrangement:an analysis of 63 cases in adults
10.13315/j.cnki.cjcep.2025.02.006
- VernacularTitle:成人伴IRF4重排的大B细胞淋巴瘤63例临床病理学特征及预后分析
- Author:
Yuxiu ZHANG
1
;
Hongmei YI
1
;
Anqi LI
1
;
Yimin LI
1
;
Binshen OUYANG
1
;
Lei DONG
1
;
Lei ZHANG
1
;
Haimin XU
1
;
Chaofu WANG
1
Author Information
1. 上海交通大学医学院附属瑞金医院病理科,上海 200025
- Publication Type:Journal Article
- Keywords:
lymphoma;
large B-cell lymphoma with IRF4 rearrangement;
diffuse large B-cell lymphoma;
fluorescence in situ hybridization
- From:
Chinese Journal of Clinical and Experimental Pathology
2025;41(2):171-178
- CountryChina
- Language:Chinese
-
Abstract:
Purpose To investigate the clinicopathological features and prognosis of adult large B-cell lymphoma with IRF4 rearrangement(LBCL-IRF4r).Methods Clinical data of 63 adult LBCL-IRF4r cases were collected.The EnVision two-step method was employed for immunohistochemical staining,and fluorescence in situ hybridization was used to detect rearrangements or deletions of the IRF4,BCL2,MYC,BCL6,and TP53 genes.The relationship be-tween clinicopathological features and prognosis was analyzed and compared with data from 132 adult non-specified dif-fuse large B-cell lymphoma(DLBCL)cases.Results Among the 63 adult LBCL-IRF4r patients,the male to female ratio was 1.1∶1,with a median age of 54.0 years(range 20-84 years),and 14 cases(22.2％)were＜40 years old,24 cases(38.1％)were between 40 and 60 years old,and 25 cases(39.7％)were＞60 years old.18 cases(28.6％)were involved in Waldeyer's ring,along with 8 cases(12.7％)in cervical lymph nodes,7 cases(11.1％)in other lymph nodes and lymphatic organs,13 cases(20.6％)in stomach,4 cases(6.4％)in intestine,and 13 cases(20.6％)in other extranodal sites.63 cases showed IRF4 rearrangements,with no BCL2 and MYC translocations(0/58),30.9％(17/55)had BCL6 translocations,and 16.3％(8/49)had TP53 deletions.59 pa-tients were followed up for a median of 28 months(range 1-65 months).48 patients(81.4％)achieved complete re-sponse,10 patients(16.9％)experienced disease progression or relapse,and 3 patients(5.1％)died.Univariate a-nalysis showed that lactate dehydrogenase level,Ann Arbor stage,international prognostic index(IPI)score,growth pattern,Hans classification,and double expression of BCL2 and C-MYC were significantly associated with progression-free survival.Age,Ann Arbor stage,and IPI score were significantly associated with overall survival.Multivariate Cox regression analysis showed that double expression of BCL2 and C-MYC was an independent prognostic factor for pro-gression-free survival.Adult LBCL-IRF4r had significantly higher complete response rate and progression-free survival than adult DLBCL.Conclusion LBCL-IRF4r occurs in adults of all age groups,commonly affecting Waldeyer's ring,cervical lymph nodes,and gastrointestinal tract,and has a favorable clinical prognosis.
