Lavergne A, Brochériou I, Delfau M H, Copie-Bergman C, Houdart R, Gaulard P H
Service Central d'Anatomie et Cytologie Pathologiques, Hôpital Lariboisière, Paris, France.
Histopathology. 1998 Mar;32(3):271-6. doi: 10.1046/j.1365-2559.1998.00394.x.
Primary intestinal T-cell lymphomas account for about 5% of all primary gastrointestinal lymphomas and are mostly associated with coeliac disease. They usually express the CD3-associated T-cell receptor alpha/beta heterodimer and HML1, and some are related with Epstein-Barr virus (EBV). As far as we know, the present report describes the first case of primary gamma-delta (gamma delta) EBV-associated intestinal T-cell lymphoma without enteropathy. Only hepatosplenic, nasal and cutaneous gamma delta T-cell lymphomas have previously been described.
Our case concerned a 43-year-old man with no history of coeliac disease, who presented with multifocal small bowel involvement showing high grade T-cell lymphoma with medium sized and large pleomorphic cells and a small pleomorphic T-cell component. Angioinvasion and angiocentricity were occasionally present. Immunohistochemical studies of lymphoma cells showed a T-cell gamma delta phenotype (CD3+, CD2+, TCR delta 1+, V delta 2+ and beta F1-) without expression of CD4, CD8, CD5, or HML1. Most tumour cells were positive for the cytotoxic granular proteins TiA1 and granzyme B. Rearrangement of the TCR gamma chain gene was demonstrated by polymerase chain reaction and in-situ hybridization with EBER probes revealed strong nuclear positivity in virtually all neoplastic cells.
We described the first case of primary intestinal gamma delta T-cell lymphoma without enteropathy in which EBV might fulfil a pathogenic role.
原发性肠道T细胞淋巴瘤约占所有原发性胃肠道淋巴瘤的5%，且大多与乳糜泻相关。它们通常表达与CD3相关的T细胞受体α/β异二聚体和HML1，部分与爱泼斯坦-巴尔病毒（EBV）有关。据我们所知，本报告描述了首例无肠病的原发性γ-δ（γδ）EBV相关肠道T细胞淋巴瘤。此前仅报道过肝脾、鼻和皮肤γδT细胞淋巴瘤。
我们的病例是一名43岁男性，无乳糜泻病史，表现为多灶性小肠受累，显示为高级别T细胞淋巴瘤，伴有中等大小和大的多形性细胞以及少量多形性T细胞成分。偶尔可见血管侵犯和血管中心性。淋巴瘤细胞的免疫组织化学研究显示为T细胞γδ表型（CD3 +、CD2 +、TCRδ1 +、Vδ2 +和βF1 -），不表达CD4、CD8、CD5或HML1。大多数肿瘤细胞对细胞毒性颗粒蛋白TiA1和颗粒酶B呈阳性。通过聚合酶链反应证实了TCRγ链基因重排，并且用EBER探针进行原位杂交显示几乎所有肿瘤细胞均有强核阳性。
我们描述了首例无肠病的原发性肠道γδT细胞淋巴瘤，其中EBV可能起致病作用。
