Department of Pathology, Faculty of Medicine, Fukuoka University, Fukuoka, Japan.
Histopathology. 2011 Feb;58(3):395-407. doi: 10.1111/j.1365-2559.2011.03768.x. Epub 2011 Feb 16.
To elucidate the clinicopathological findings of primary intestinal enteropathy-associated T cell lymphoma (EATL) in Japan, a non-endemic area for coeliac disease.
Of the 24 cases, four (17%) had large-cell lymphoma (type I), and the remaining 20 (83%) had medium-sized lymphoma (type II). Lymphoma cells of the three type I cases were CD56-positive. Only one (4%) case showed typical CD56- and CD8-negative and CD30-positive type I EATL. In type II EATL, lymphoma cells of the 16 (80%) and 11 (55%) cases were positive for CD56 and CD8, respectively. Intramucosal tumour spreading and adjacent enteropathy-like lesions were detected in 15 (71%) and 16 (76%) of 21 cases, with a severe increase of intraepithelial lymphocytes (IELs) in 12 (57%). IELs of enteropathy-like lesions in five (24%) cases expressed T-bet, with no cases of CD30-positive IELs. Characteristic findings from comparative genomic hybridization of 15 cases indicated gains of 8q2 (47%), Xp (53%) and Xq (73%), but no gain of 9q3. Regarding, human leucocyte antigen (HLA) status, six cases examined did not express the DQB1*02 allele.
Japanese EATL exhibited different histology, cytogenetic findings and HLA status from those of typical type I EATL. The rare incidence of coeliac disease may influence the tumour cell characteristics of EATL and IELs.
阐明日本（一个非乳糜泻流行地区）原发性肠道肠病相关 T 细胞淋巴瘤（EATL）的临床病理特征。
24 例中，4 例（17%）为大细胞淋巴瘤（I 型），其余 20 例（83%）为中细胞淋巴瘤（II 型）。3 例 I 型病例的淋巴瘤细胞 CD56 阳性。仅 1 例（4%）表现为典型的 CD56-和 CD8-、CD30 阳性的 I 型 EATL。在 II 型 EATL 中，16 例（80%）和 11 例（55%）的淋巴瘤细胞分别 CD56 和 CD8 阳性。21 例中有 15 例（71%）和 16 例（76%）检测到黏膜内肿瘤扩散和相邻的肠病样病变，12 例（57%）有上皮内淋巴细胞（IEL）显著增加。5 例（24%）肠病样病变的 IEL 表达 T-bet，无 CD30 阳性 IEL。15 例比较基因组杂交的特征性发现表明 8q2（47%）、Xp（53%）和 Xq（73%）的获得，但没有 9q3 的获得。关于人类白细胞抗原（HLA）状态，6 例检查均未表达 DQB1*02 等位基因。
日本 EATL 的组织学、细胞遗传学表现和 HLA 状态与典型的 I 型 EATL 不同。乳糜泻发病率低可能影响 EATL 和 IEL 的肿瘤细胞特征。
