摘要
目的探讨成人伴IRF4重排的大B细胞淋巴瘤(large B-cell lymphoma with IRF4 rearrangement,LBCL-IRF4r)的临床病理学特征及预后。方法收集63例成人LBCL-IRF4r的临床资料,采用免疫组化EnVision两步法检测相关蛋白表达,FISH技术检测IRF4、BCL2、MYC、BCL6和TP53基因重排或缺失,分析临床病理特征与预后的关系。另收集132例成人非特指型弥漫大B细胞淋巴瘤(diffuse large B-cell lymphoma,DLBCL)预后数据进行对比分析。结果63例成人LBCL-IRF4r男女比为1.1∶1,中位年龄54.0岁(范围20~84岁),其中<40岁14例(22.2%)、40~60岁24例(38.1%)、>60岁25例(39.7%);Waldeyer环18例(28.6%)、颈部淋巴结8例(12.7%)、其他淋巴结和淋巴器官合计7例(11.1%)、胃13例(20.6%)、肠4例(6.4%)和其他结外部位合计13例(20.6%);63例均存在IRF4基因重排,无BCL2和MYC基因易位(0/58),30.9%(17/55)存在BCL6基因易位,16.3%(8/49)有TP53基因缺失。59例获得随访,中位随访28个月(范围1~65个月),完全缓解48例(81.4%),疾病进展或复发10例(16.9%),死亡3例(5.1%)。单因素分析显示,乳酸脱氢酶水平、Ann Arbor分期、国际预后指数(international prognostic index,IPI)评分、生长模式、Hans分型和双表达BCL2和C-MYC与患者无进展生存显著相关;年龄、Ann Arbor分期和IPI评分与总生存显著相关。多因素Cox回归分析显示,双表达BCL2和C-MYC是无进展生存的独立预后因素。成人LBCL-IRF4r的完全缓解率和无进展生存显著高于成人非特指型DLBCL。结论LBCL-IRF4r可发生于成人各年龄段,好发于Waldeyer环、颈部淋巴结和胃肠道,临床预后较好。
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 between clinicopathological features and prognosis was analyzed and compared with data from 132 adult non-specified diffuse 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 patients were followed up for a median of 28 months(range 1-65 months).48 patients(81.4%)achieved complete response,10 patients(16.9%)experienced disease progression or relapse,and 3 patients(5.1%)died.Univariate analysis 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 progression-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.
作者
张瑜琇
易红梅
李安琪
李医民
欧阳斌燊
董磊
张蕾
许海敏
王朝夫
Zhang Yuxiu;Yi Hongmei;Li Anqi;Li Yimin;Ouyang Binshen;Dong Lei;Zhang Lei;Xu Haimin;Wang Chaofu(Department of Pathology,Ruijin Hospital Affiliated to Shanghai Jiaotong University School of Medicine,Shanghai 200025,China)
出处
《临床与实验病理学杂志》
北大核心
2025年第2期171-178,共8页
Chinese Journal of Clinical and Experimental Pathology
基金
国家自然科学基金(82300212)
上海市科技委科研计划项目(21ZR1440100)。
关键词
淋巴瘤
伴IRF4重排的大B细胞淋巴瘤
弥漫大B细胞淋巴瘤
FISH
lymphoma
large B-cell lymphoma with IRF4 rearrangement
diffuse large B-cell lymphoma
fluorescence in situ hybridization
