Journal
LEUKEMIA
Volume 27, Issue 8, Pages 1688-1696Publisher
NATURE PUBLISHING GROUP
DOI: 10.1038/leu.2013.41
Keywords
enteropathy-associated T-cell lymphoma; epitheliotropism; enteropathy; pathology; lymphoma; CD8 alpha alpha
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Funding
- National Medical Research Council of Singapore [2010/022]
- Singhealth Foundation [SHF/FG438P/2010]
- HSBC Trustee (Singapore) Limited
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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.
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