脾脏滤泡性树突状细胞肿瘤伴弥漫性大B细胞淋巴瘤

Follicular dendritic cell tumor of the spleen associated with diffuse large B-cell lymphoma.

作者信息

Laurent Camille, Meggetto Fabienne, de Paiva Geisilene Russano, Selves Janick, Palasse Julien, Laurent Guy, Brousset Pierre

机构信息

INSERM, U.563, Centre de Physiopathologie de Toulouse-Purpan, Toulouse F-31300, France; Université Paul-Sabatier, Toulouse F-31400, France.

出版信息

Hum Pathol. 2008 May;39(5):776-80. doi: 10.1016/j.humpath.2007.08.022. Epub 2008 Mar 4.

DOI:10.1016/j.humpath.2007.08.022

PMID:18291441

Abstract

We report the case of a 50-year-old woman with an isolated large mass of the lower pole of the spleen. Splenectomy was performed and revealed a follicular dendritic cell (FDC) tumor associated with diffuse large cell lymphoma. Dendritic cells were CD21(+), CD35(+), CNA42(+), CD20(-), and Epstein-Barr virus (EBV)(+). They contained a clonal EBV genome as shown by polymerase chain reaction analysis of the LMP-1 gene polymorphism. Interestingly, the lymphoma cells were intermingled with the neoplastic FDCs and displayed a germinal center cell phenotype (CD20(+), CD10(+), Bcl2(+), and EBV(-)). Double staining confirmed that EBV was restricted to the FDCs. Clinical, radiologic, and pathologic staging showed no other lymphoma localization. To the best of our knowledge, this association has never been reported. Based on the well-established role of FDCs in B-cell survival and proliferation, this observation suggests that the FDC tumor represented a favorable microenvironment for lymphoma cells with germinal center phenotype.

摘要

我们报告了一例50岁女性，其脾脏下极有一个孤立的大肿块。行脾切除术，结果显示为与弥漫性大细胞淋巴瘤相关的滤泡树突状细胞（FDC）肿瘤。树突状细胞CD21(+)、CD35(+)、CNA42(+)、CD20(-)，且爱泼斯坦-巴尔病毒（EBV）(+)。通过对LMP-1基因多态性的聚合酶链反应分析表明，它们含有克隆性EBV基因组。有趣的是，淋巴瘤细胞与肿瘤性FDCs混合存在，并表现出生发中心细胞表型（CD20(+)、CD10(+)、Bcl2(+)，且EBV(-)）。双重染色证实EBV仅限于FDCs。临床、影像学和病理分期均未显示其他淋巴瘤定位。据我们所知，这种关联从未有过报道。基于FDCs在B细胞存活和增殖中已明确的作用，这一观察结果表明，FDC肿瘤代表了具有生发中心表型的淋巴瘤细胞的有利微环境。