淋巴结滤泡辅助T细胞淋巴瘤滤泡型7例临床病理学分析  

Nodal follicular helper T-cell lymphoma,follicular type:a clinicopathological analysis of 7 cases

作　　者：汤汪洋 王健超 钟礼花[2] 张文芳 朱琼 陈燕坪[2] 陈刚[2] Tang Wangyang;Wang Jianchao;Zhong Lihua;Zhang Wenfang;Zhu Qiong;Chen Yanping;Chen Gang(Department of Pathology,Sir Runrun Shaw Hospital,School of Medicine,Zhejiang University,Hangzhou 310000,China;Department of Pathology,Fujian Medical University Cancer Hospital,Fujian Cancer Hospital,Fuzhou 350014,China)

机构地区：[1]浙江大学医学院附属邵逸夫医院病理科,杭州310000 [2]福建医科大学附属肿瘤医院/福建省肿瘤医院病理科,福州350014

出　　处：《临床与实验病理学杂志》2025年第2期191-197,共7页Chinese Journal of Clinical and Experimental Pathology

基　　金：福建省科技厅科技创新联合基金(2021Y9215)。

摘　　要：目的探讨淋巴结滤泡辅助T细胞淋巴瘤滤泡型(nodal follicular helper T-cell lymphoma,follicular type,nTFHL-F)的临床病理学特征。方法收集7例nTFHL-F的临床资料,总结组织学形态,进行免疫组化染色及分子检测,并复习相关文献。结果男女比为6∶1,中位年龄62岁。首发症状:6例为颈部淋巴结肿大,1例腹胀不适;临床分期:进展期6例,局限期1例。肿瘤呈滤泡结节状生长,无显著的多形性炎症背景及高内皮血管增生。其中5例呈进行性转化的生发中心(progression transformer germinal center,PTGC)样生长模式,2例滤泡性淋巴瘤(follicular lymphoma,FL)样生长模式。肿瘤细胞可表现为中心细胞样、单核B细胞样、胞质透亮水肿样3种形态。肿瘤细胞表达3种以上滤泡辅助T细胞标志物。FISH检测结果显示ITK∷SYK基因融合皆阴性(0/7),二代测序检出TET2基因突变2例(2/2),RHOA基因突变1例(1/2),VAV1基因突变1例(1/2)。随访2~64个月,3例死亡(3/7),中位生存时间37个月。结论nTFHL-F好发于中老年男性,临床分期高,预后较差。nTFHL-F与淋巴结滤泡辅助T细胞淋巴瘤血管免疫母型关系密切,可在同一个体中同时发生。Purpose This study aims to analyze the clinical,pathological,and molecular genetic characteristics of nodal follicular helper T-cell lymphoma,follicular type(nTFHL-F).Methods 7 cases of nTFHL-F were reviewed.Clinical data were collected,tissue morphology was summarized,and immunohistochemical staining and molecular testing were performed.Results The median age of patients was 62 years with a male-to-female ratio of 6∶1.The initial symptoms included neck lymphadenectasis in 6 cases and abdominal discomfort in one.Six cases were in advanced stages,while 1 case was in the localized stage.The tumors exhibited a vague,irregular follicular nodular pattern,without significant polymorphic inflammatory background or high endothelial vascular proliferation.Five cases showed a progressive transformation resembling germinal center pattern,and two cases exhibited a follicular lymphoma-like growth pattern.Tumor cells presented three distinct morphologies:centrocyte-like appearance,monocytoid B cell-like appearance,and atypical cells with abundant,transparent cytoplasm.Tumor cells expressed at least three follicular T-cell markers.Testing for ITK∷SYK gene fusion was negative in all cases(0/7).Next generation sequencing identified mutations in TET2 gene in two cases(2/2),the RHOA gene in one case(1/2),and VAV1 gene in one case(1/2).The follow-up duration ranged from 2 to 64 months,with three deaths(3/7),and a median survival time of 37 months.Conclusion nTFHL-F predominantly occurs in middle-aged to elderly males,presenting with advanced clinical stages,and has a poor prognosis.nTFHL-F is closely associated with nodal follicular helper T-cell lymphoma,angioimmunoblastic type,and mya coexist within the same individual.

关 键 词：淋巴瘤 淋巴结滤泡辅助T细胞淋巴瘤 滤泡型 血管免疫母型 

分 类 号：R733.4[医药卫生—肿瘤]