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肠病型T细胞淋巴瘤

Enteropathy-type T-cell lymphoma.

作者信息

Zettl Andreas, deLeeuw Ron, Haralambieva Eugenia, Mueller-Hermelink Hans-Konrad

机构信息

The Institute of Pathology, University of Würzburg, Würzburg, Germany.

出版信息

Am J Clin Pathol. 2007 May;127(5):701-6. doi: 10.1309/nw2bk1dxb0eqg55h.

Abstract

Session 7 of the Society for Hematopathology/European Association for Haematopathology Workshop was devoted to case presentations and discussion of enteropathy-type T-cell lymphoma (ETL) and other T-cell lymphomas involving the gastrointestinal tract. ETL is a rare type of T-cell lymphoma, often associated with a history of celiac disease, that usually arises in the jejunum but can involve other gastrointestinal tract sites (eg, stomach and colon). As the cases submitted illustrate, there are 2 histologic groups of ETL that correlate with clinical and immunophenotypic features. Pleomorphic-anaplastic ETL is usually associated with a history of celiac disease and histologic evidence of enteropathy and is most often CD56-. Monomorphic ETL often occurs without a history of celiac disease, has variable histologic evidence of enteropathy, and is usually CD56+. Comparative genomic hybridization has shown recurrent chromosomal gains and losses that are characteristic of ETL and uncommon in other T-cell lymphomas, providing useful ancillary data for the diagnosis of ETL.

摘要

血液病理学学会/欧洲血液病理学协会研讨会的第7场会议专门进行了病例展示,并对肠病型T细胞淋巴瘤(ETL)及其他累及胃肠道的T细胞淋巴瘤展开了讨论。ETL是一种罕见的T细胞淋巴瘤,常与乳糜泻病史相关,通常起源于空肠,但也可累及其他胃肠道部位(如胃和结肠)。正如提交的病例所示,ETL有两个组织学类型,与临床和免疫表型特征相关。多形性-间变性ETL通常与乳糜泻病史及肠病的组织学证据相关,且大多为CD56阴性。单形性ETL常无乳糜泻病史,肠病的组织学证据各异,且通常为CD56阳性。比较基因组杂交显示,ETL存在特征性的反复染色体增减,而在其他T细胞淋巴瘤中并不常见,这为ETL的诊断提供了有用的辅助数据。

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