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Title: Type II EATL (epitheliotropic intestinal T-cell lymphoma): A neoplasm of intra-epithelial T-cells with predominant CD8αα phenotype
Authors: Tan, S.-Y. 
Chuang, S.-S.
Tang, T.
Tan, L.
Ko, Y.-H.
Chuah, K.-L.
Ng, S.-B. 
Chng, W.-J. 
Gatter, K.
Loong, F.
Liu, Y.-H.
Hosking, P.
Cheah, P.-L.
Teh, B.-T. 
Tay, K.
Koh, M.
Lim, S.-T. 
Keywords: CD8αα
enteropathy-associated T-cell lymphoma
Issue Date: Aug-2013
Citation: Tan, S.-Y., Chuang, S.-S., Tang, T., Tan, L., Ko, Y.-H., Chuah, K.-L., Ng, S.-B., Chng, W.-J., Gatter, K., Loong, F., Liu, Y.-H., Hosking, P., Cheah, P.-L., Teh, B.-T., Tay, K., Koh, M., Lim, S.-T. (2013-08). Type II EATL (epitheliotropic intestinal T-cell lymphoma): A neoplasm of intra-epithelial T-cells with predominant CD8αα phenotype. Leukemia 27 (8) : 1688-1696. ScholarBank@NUS Repository.
Abstract: 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 TCRαβ over TCRγδ (1.6:1). In keeping with the normal ratio of IEL subsets, CD8 + cases showed predominant CD8αα 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. © 2013 Macmillan Publishers Limited. All rights reserved.
Source Title: Leukemia
ISSN: 08876924
DOI: 10.1038/leu.2013.41
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