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Potentially important miRNAs in enteropathy-associated T-cell lymphoma pathogenesis: A pilot study

机译:潜在的重要miRNA在肠病相关T细胞淋巴瘤发病机理中的初步研究

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Enteropathy associated T-cell lymphoma (EATL) is a rare form of NonHodgkin Lymphoma occurring primarily in the intestinal tract and whicharises from intra-epithelial T-lymphocytes (IELs) [1]. Previously known asType 1 EATL, this lymphoma has a strong association with coeliac diseaseand occurs with a higher frequency in Northern Europe where coeliac disease is most prevalent. Morphologically the lymphoma cells primarilyconsist of medium-sized to large tumour cells with round or angulated vesicular nuclei, prominent nucleoli, and pale-staining cytoplasm and is oftenassociated with a moderate to abundant reactive infltrate of eosinophils,histiocytes, and small lymphocytes [2]. These lymphomas are characteristically CD56 negative but may express CD30. Prior genetic studies haveshown that homozygosity for HLA-DQ2 (HLA-DB1*02) and allelic variantsof the MYO9B gene region maybe associated with previously classifedEATL and indeed, data from comparative genomic hybridization studies ontumour DNA suggest that chromosomal gains of 1q and 5q and segmentalamplifcation of 9q or deletion in 16q are important in EATL pathogenesis[2]. Following the new WHO classifcation of lymphoid neoplasms Type 2EATL has been reclassifed as monomorphic epitheliotropic intestinal T-celllymphoma (MEITL) [1]. MEITL is less commonly associated with coeliacdisease and is characterized by a monomorphic infltrate of small- tomedium-sized lymphoma cells. CD30 is often negative in MEITL, and CD56positivity suggests that a di?erent mechanism underlies the lymphomagenicprocess of this tumour in contrast to EATL [2].
机译:肠病相关的T细胞淋巴瘤(EATL)是一种非霍奇金淋巴瘤的罕见形式,主要发生在肠道,其上皮内T淋巴细胞(IEL)引起[1]。这种淋巴瘤以前被称为1型EATL,与乳糜泻有很强的联系,在乳糜泻最盛行的北欧,发生频率更高。从形态学上讲,淋巴瘤细胞主要由中等大小的肿瘤细胞组成,具有圆形或成角的囊泡核,突出的核仁和浅色的细胞质,并经常与嗜酸性粒细胞,组织细胞和小淋巴细胞的中度至大量反应性浸润有关[2]。这些淋巴瘤的特征是CD56阴性,但可能表达CD30。先前的遗传研究表明,HLA-DQ2(HLA-DB1 * 02)和MYO9B基因区域的等位基因变体的纯合性可能与先前分类的EATL有关,实际上,来自比较基因组杂交研究的肿瘤DNA数据表明,1q和5q的染色体增益以及节段扩增9q的缺失或16q的缺失在EATL发病机制中很重要[2]。根据WHO对淋巴瘤的新分类,2EATL型已被重新分类为单形上皮性肠T细胞淋巴瘤(MEITL)[1]。 MEITL很少与腔肠酸酶相关,其特征是小中型淋巴瘤细胞的单形浸润。 CD30在MEITL中通常是阴性的,与EATL相比,CD56阳性表明该肿瘤的淋巴瘤形成过程是不同的机制[2]。

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