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Enteropathy-Type T-Cell Lymphoma

Andreas Zettl MD, Ron deLeeuw, Eugenia Haralambieva MD, Hans-Konrad Mueller-Hermelink MD
DOI: http://dx.doi.org/10.1309/NW2BK1DXB0EQG55H 701-706 First published online: 1 May 2007

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.

Key Words:
  • Enteropathy
  • Celiac disease
  • T-cell lymphoma
  • Intestine
  • Comparative genomic hybridization
  • 9q33-q34 gains