Type II EATL (epitheliotropic intestinal T-cell lymphoma): a neoplasm of intra-epithelial T-cells with predominant CD8αα phenotype
Type II EATL (epitheliotropic intestinal T-cell lymphoma): a neoplasm of intra-epithelial T-cells with predominant CD8αα phenotype
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DOI：
10.1038/leu.2013.41
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发表时间：
2013-08-01
期刊：
影响因子：
11.4
通讯作者：
Lim, S-T
中科院分区：
文献类型：
--
作者：
Tan, S-Y;Chuang, S-S;Lim, S-T
在这项多中心研究中，我们检查了60例II型肠病相关的T细胞淋巴瘤（EATL）从亚太地区的组织学审查，免疫组化和分子技术。患者大多为成年男性（中位年龄：58岁，男：女2.6：1），表现为腹痛（60%）、肠穿孔（40%）和体重减轻（28%）。无腹腔疾病史，中位生存期仅为7个月。在组织学上，这些肿瘤可分为（i）中央肿瘤区，包括单一的肿瘤淋巴细胞群，（ii）外周区，特征为发育不良的绒毛和形态学非典型淋巴细胞，显示向上皮性，（iii）远端粘膜，具有正常的绒毛结构和细胞学正常的上皮内淋巴细胞（IEL）。以巨核细胞相关酪氨酸激酶（MATK）（87%）的广泛核表达和通常的CD8（+）CD56（+）（88%）细胞毒性表型为特征，CD20（24%）频繁异常表达。在40%的患者中，T细胞受体（TCR）表达沉默或不可评估，但在其余患者中，TCR α β的表达高于TCR β δ（1.6：1）。与IEL亚群的正常比例保持一致，CD8（+）病例显示主要的CD8 α α同源二聚体表达（77%），与TCR谱系无关。这些肿瘤构成了一个独特的实体，从经典的EATL，病理学可能反映肿瘤进展的IEL前体，其残留物往往是在远处的粘膜。
In this multicentre study, we examined 60 cases of Type II enteropathy-associated T-cell lymphoma (EATL) from the Asia-Pacific region by histological review, immunohistochemistry and molecular techniques. Patients were mostly adult males (median age: 58 years, male: female 2.6:1), presenting with abdominal pain (60%), intestinal perforation (40%) and weight loss (28%). None had a history of coeliac disease and the median survival was only 7 months. Histologically, these tumours could be divided into (i) central tumour zone comprising a monotonous population of neoplastic lymphocytes, (ii) peripheral zone featuring stunted villi and morphologically atypical lymphocytes showing epitheliotropism, and (iii) distant mucosa with normal villous architecture and cytologically normal intra-epithelial lymphocytes (IELs). Characterized by extensive nuclear expression of Megakaryocyte-associated tyrosine kinase (MATK) (87%) and usually a CD8(+)CD56(+) (88%) cytotoxic phenotype, there was frequent aberrant expression of CD20 (24%). T-cell receptor (TCR) expression was silent or not evaluable in 40% but of the remainder, there was predominant expression of TCR alpha beta over TCRg delta (1.6:1). In keeping with the normal ratio of IEL subsets, CD8(+) cases showed predominant CD8 alpha alpha homodimer expression (77%), regardless of TCR lineage. These tumours constitute a distinct entity from classical EATL, and the pathology may reflect tumour progression from IEL precursors, remnants of which are often seen in the distant mucosa.