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CD5阳性弥漫性大B细胞淋巴瘤一个亚群的滤泡起源

Follicular origin of a subset of CD5+ diffuse large B-cell lymphomas.

作者信息

Manazza Andrea D, Bonello Lisa, Pagano Marco, Chiusa Luigi, Novero Domenico, Stacchini Alessandra, Martini Giuseppe, Vitolo Umberto, Tarella Corrado, Inghirami Giorgio, Palestro Giorgio, Chiarle Roberto

机构信息

Center for Experimental Research and Medical Studies and Department of Biomedical Sciences and Human Oncology, University of Turin, Turin, Italy.

出版信息

Am J Clin Pathol. 2005 Aug;124(2):182-90. doi: 10.1309/8EX5-B9JF-HPEK-82BU.

Abstract

Most follicular lymphomas (FLs) have a phenotype consistent with the origin from CD5-, CD10+, bcl-6+ follicular center cells and can progress to diffuse large B-cell lymphoma (DLBCL). CD5 is expressed in about 10% of DLBCLs, showing prognostic value, whereas expression is rare in FL. We present 6 cases with coexisting features of CD5+ FL and CD5+ DLBCL, supporting a follicular origin for some CD5+ DLBCLs. The follicular areas showed a meshwork of CD21+ follicular dendritic cells that were lacking in the DLBCL areas. All cases showed a clonal CD19+, CD20+, CD5+, and CD10+ population in both follicular and diffuse areas. Molecularly, 4 of 6 cases demonstrated immunoglobulin heavy chain rearrangements and 1 case, a bcl-2/immunoglobulin heavy chain gene rearrangement. Somatic hypermutations were high in all 4 cases, in keeping with their germinal center origin. Four of five patients died of disease within 42 months, consistent with the proposed prognostic value of CD5 expression in DLBCL. Our data describe an aggressive variant of CD5+ FL suggesting the follicular origin of some CD5+ DLBCLs.

摘要

大多数滤泡性淋巴瘤(FL)具有与源自CD5阴性、CD10阳性、bcl-6阳性滤泡中心细胞一致的表型,并且可进展为弥漫性大B细胞淋巴瘤(DLBCL)。CD5在约10%的DLBCL中表达,具有预后价值,而在FL中表达罕见。我们报告了6例同时具有CD5阳性FL和CD5阳性DLBCL特征的病例,支持某些CD5阳性DLBCL起源于滤泡。滤泡区域显示出CD21阳性滤泡树突状细胞的网络结构,而DLBCL区域则缺乏这种结构。所有病例在滤泡和弥漫区域均显示出克隆性CD19阳性、CD20阳性、CD5阳性和CD10阳性细胞群。分子层面上,6例中有4例显示免疫球蛋白重链重排,1例显示bcl-2/免疫球蛋白重链基因重排。所有4例的体细胞超突变率都很高,与其生发中心起源相符。5例患者中有4例在42个月内死于疾病,这与DLBCL中CD5表达所提示的预后价值一致。我们的数据描述了一种侵袭性的CD5阳性FL变体,提示某些CD5阳性DLBCL起源于滤泡。

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