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一名日本患者出现的伴有 MYC 易位的非典型肠病相关 T 细胞淋巴瘤:病例报告。

An unusual enteropathy-associated T-cell lymphoma with MYC translocation arising in a Japanese patient: a case report.

机构信息

Department of Surgery, Nippon Steel Yawata Memorial Hospital, Kitakyushu 805-8508, Japan.

出版信息

World J Gastroenterol. 2012 May 21;18(19):2434-7. doi: 10.3748/wjg.v18.i19.2434.

Abstract

Enteropathy-associated T-cell lymphoma (EATL) is a rare peripheral T-cell lymphoma classified into 2 types, with or without celiac disease, based on histology. Type 2 EATL is less commonly associated with celiac disease, in which cells are characterized by being monomorphic and small- to medium-sized. Cells are characterized by CD8 and CD56 expression and c-MYC oncogene locus gain. We present an atypical case of type 2 EATL in the jejunum, with human T-lymphotropic virus-1 that was CD4- CD8+ CD56- CD30- CD25- TIA-1+ and granzyme B+ on immunohistological staining. It also displayed translocation of chromosome 8p24 (c-MYC), as determined by fluorescent in situ hybridization. Mucosal spreading and intraepithelial invasion by lymphoma with villous atrophy were detected adjacent to the mucosal layer. The lymphoma may be derived from intraepithelial CD8+ T cells, similar to celiac disease.

摘要

肠病相关 T 细胞淋巴瘤(EATL)是一种罕见的外周 T 细胞淋巴瘤,根据组织学可分为有或无乳糜泻两种类型。2 型 EATL 与乳糜泻的关联较少,其特征为细胞形态单一且体积小至中等。细胞的特征是 CD8 和 CD56 表达以及 c-MYC 癌基因座获得。我们报告了一例小肠 2 型 EATL 的不典型病例,人类 T 淋巴细胞病毒-1 呈 CD4-CD8+CD56-CD30-CD25-TIA-1+和 granzyme B+,免疫组织化学染色阳性。荧光原位杂交还显示染色体 8p24(c-MYC)易位。在黏膜层附近检测到淋巴瘤的黏膜扩散和上皮内浸润,伴有绒毛萎缩。这种淋巴瘤可能来源于上皮内 CD8+T 细胞,类似于乳糜泻。

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