淋巴结滤泡辅助T细胞淋巴瘤伴B细胞克隆性增生10例临床病理特征分析  

Nodal follicular helper T cell lymphoma with monoclonal B-cell hyperplasia:a clinicopathologic study of ten cases

作　　者：王玥[1] 郭亮[1] 金春卉 曲丽梅[1] WANG Yue;GUO Liang;JIN Chunhui;QU Limei(Department of Pathology,the First Hospital of Jilin University,Changchun 130001,China)

机构地区：[1]吉林大学第一医院病理科,长春130001

出　　处：《临床与实验病理学杂志》2024年第10期1068-1074,共7页Chinese Journal of Clinical and Experimental Pathology

基　　金：吉林省科技发展计划项目(20230401090YY)。

摘　　要：目的探讨淋巴结滤泡辅助T细胞淋巴瘤血管免疫母细胞型(nodal follicular helper T cell lymphoma,angioimmunoblastic-type,nTFHL-AI)伴B细胞克隆性增生的临床病理学特征、免疫表型、分子特征、治疗及预后。方法收集10例nTFHL-AI伴B细胞克隆性增生患者的临床病理资料,分别行HE、免疫组化和基因重排检测,并复习相关文献。结果10例患者中男性5例,女性5例,中位年龄73岁。临床表现以全身淋巴结肿大、脾肿大及B症状为主;Ann Arbor分期:Ⅳ期8例,Ⅰ+Ⅱ期2例。实验室检查以β2微球蛋白和乳酸脱氢酶(LDH)升高,血红蛋白、红细胞和血小板减少为主,血浆EBV核酸定量阳性8例。镜下均可见典型结节状聚集或散在的透明细胞、分枝状高内皮血管及增生杂乱“风吹”状的滤泡树突网。肿瘤细胞背景中嗜酸性粒细胞浸润0~5个/HPF 7例,5~10个/HPF 2例,>50个/HPF 1例。浆细胞含量≤5%6例,浆细胞含量10%和20%各1例,浆细胞含量较多(30%)2例。组织细胞明显增生7例。见RS样大细胞1例。背景中含大量B细胞5例,含少量B细胞5例。10例患者均表达T细胞标志物,滤泡辅助性T细胞标志物CD10、BCL6、CXCL13和PD-1同时阳性6例,BCL6、CXCL13和PD-1同时阳性10例。EBER原位杂交阳性8例。10例患者均检测到TCR基因重排和IG基因重排阳性。所有患者诊断后均行化疗,其中3例疾病进展死亡。结论伴B细胞克隆性增生nTFHL-AI中,B细胞的克隆性增生与EBV是否感染及感染细胞的数量无关,同时浆细胞增生提示预后不良。Purpose To investigate the clinicopathological features,immunophenotypes,molecular characteristics,treatment and prognosis of nodal follicular helper T cell lymphoma,angioimmunoblastic type(nTFHL-AI)with B cell clonal hyperplasia.Methods The clinicopathological data of 10 nTFHL-AI patients with B cell clonal hyperplasia were collected from medical records,with HE and immunohistochemical staining and gene rearrangement analysis.Related literature was also reviewed.Results The included 10 patients were 5 males and 5 females with a median age of 73 years.The clinical manifestations were mainly systemic lymphadenopathy,splenomegaly and B symptoms.8 patients were categorized as stageⅣand 2 patients were staged asⅠ+Ⅱaccording to Ann Arbor staging criteria.Major laboratory results were increasedβ2 microglobulin,lactate dehydrogenase and decreased hemoglobin,erythrocytes and thrombocytes.Plasma Epstein-Barr virus(EBV)nucleic acid quantification was positive in 8 cases.Microscopically,the morphological patterns were nodular aggregation or scattered clear cells,branched high endothelial vessels and disorderly“wind-blown”like follicular dendritic cells(FDC).The number of infiltrated eosinophils was 0-5/HPF in 7 cases,5-10/HPF in 2 cases,and>50/HPF in 1 case.Plasma cell count was≤5%in 6 cases,10%in 1 case,20%in 1 case,and the rest 2 had relative higher count of 30%.The tumor cells of 7 cases coexisted with marked hyperplasia of histiocytes,and in only one case Reed-Sternberg(RS)-like large cells were found 5 cases showed abundant background B cells while the other 5 cases had limited background B cells.All tumor cells expressed T cell markers,6 were positive for CD10,BCL6,CXCL13 and PD-1 simultaneously and all of 10 were positive for BCL6,CXCL13 and PD-1.8 cases were positive for EBV-encoded small RNA(EBER)by in situ hybridization.Clonal TCR gene rearrangements and IG gene rearrangement were detected in all 10 patients.After diagnosis,all patients were treated with chemotherapy,and three of them died due to dise

关 键 词：淋巴结滤泡辅助T细胞淋巴瘤 血管免疫母细胞型 单克隆B细胞增生 EBV感染 肿瘤微环境 预后 

分 类 号：R733.4[医药卫生—肿瘤]