Enteropathy-Type T-Cell Lymphoma

Abstract

Session 7 of the Society for Hematopathology/European Association for Haematopathology Workshop was devoted to case presentations and discussion of enteropathy-type T-cell lymphoma (ETL) and other T-cell lymphomas involving the gastrointestinal tract. ETL is a rare type of T-cell lymphoma, often associated with a history of celiac disease, that usually arises in the jejunum but can involve other gastrointestinal tract sites (eg, stomach and colon). As the cases submitted illustrate, there are 2 histologic groups of ETL that correlate with clinical and immunophenotypic features. Pleomorphic-anaplastic ETL is usually associated with a history of celiac disease and histologic evidence of enteropathy and is most often CD56-. Monomorphic ETL often occurs without a history of celiac disease, has variable histologic evidence of enteropathy, and is usually CD56+. Comparative genomic hybridization has shown recurrent chromosomal gains and losses that are characteristic of ETL and uncommon in other T-cell lymphomas, providing useful ancillary data for the diagnosis of ETL.

Primary intestinal T-cell and NK-cell lymphomas: a clinicopathological and molecular study from China focused on type II enteropathy-associated T-cell lymphoma and primary intestinal NK-cell lymphoma

“…Classical (type I) enteropathy-associated T-cell lymphoma in the West occurs most commonly in the small intestine and less frequently in the colon or stomach. 21 However, in our study, only two of seven type II enteropathy-associated T-cell lymphoma cases were confined to the small intestine. Two occurred in the colon, two occurred both in the small intestine and colon, and one involved the stomach, small intestine, and colon non-contiguously.…”

“…In previous studies, about 90 and 80% of type II enteropathy-associated T-cell lymphomas were positive for CD56 and CD8, respectively, 21 and most intestinal NK-like T-cell lymphomas showed CD8 expression. 16,23 In our study, the rates of CD56 expression in primary intestinal NK-cell lymphomas, type II enteropathy-associated T-cell lymphomas, and peripheral T-cell lymphoma NOS were 83, 100, and 20%, respectively.…”

Enteropathy Associated T Cell Lymphoma – A Case Report of An Uncommon Extranodal T Cell Lymphoma

“…Tumour is usually multifocal and forms ulcerating nodules, plaques, strictures or less commonly large masses. The mesentry is often infiltrated and mesentric lymph nodes are commonly involved [1,2].…”

Malignancy in adult celiac disease

“…In a subset of patients that seem clinically refractory to a gluten-free diet, intra-epithelial lymphocytes have a different form of T-cell phenotypic expression: CD3 shows intra-cytoplasmic expression while CD8 expression is absent. Some believe this may reflect a specific form of refractory celiac disease (type 2) with a poor prognosis and a possible precursor lesion for the development of lymphoma [12][13][14][15] . Even lymphomas with T-cell immunophenotypic features have been detected in extra-intestinal sites, which complicates the clinical course of celiac disease.…”