title: Primary intestinal gamma-delta T-cell lymphoma with evidence of Epstein-Barr virus.
abstract: OBJECTIVE
  Primary intestinal T-cell lymphomas account for about 5% of all primary gastrointestinal lymphomas and are mostly associated with coeliac disease. They usually express the CD3-associated T-cell receptor alpha/beta heterodimer and HML1, and some are related with Epstein-Barr virus (EBV). As far as we know, the present report describes the first case of primary gamma-delta (gamma delta) EBV-associated intestinal T-cell lymphoma without enteropathy. Only hepatosplenic, nasal and cutaneous gamma delta T-cell lymphomas have previously been described.
  
  
  RESULTS
  Our case concerned a 43-year-old man with no history of coeliac disease, who presented with multifocal small bowel involvement showing high grade T-cell lymphoma with medium sized and large pleomorphic cells and a small pleomorphic T-cell component. Angioinvasion and angiocentricity were occasionally present. Immunohistochemical studies of lymphoma cells showed a T-cell gamma delta phenotype (CD3+, CD2+, TCR delta 1+, V delta 2+ and beta F1-) without expression of CD4, CD8, CD5, or HML1. Most tumour cells were positive for the cytotoxic granular proteins TiA1 and granzyme B. Rearrangement of the TCR gamma chain gene was demonstrated by polymerase chain reaction and in-situ hybridization with EBER probes revealed strong nuclear positivity in virtually all neoplastic cells.
  
  
  CONCLUSIONS
  We described the first case of primary intestinal gamma delta T-cell lymphoma without enteropathy in which EBV might fulfil a pathogenic role.
authors.affiliation: Service Central d'Anatomie et Cytologie Pathologiques, Hôpital Lariboisière, Paris, France.
authors.forename: A
authors.initials: A
authors.lastname: Lavergne
authors.forename: I
authors.initials: I
authors.lastname: Brochériou
authors.forename: M H
authors.initials: MH
authors.lastname: Delfau
authors.forename: C
authors.initials: C
authors.lastname: Copie-Bergman
authors.forename: R
authors.initials: R
authors.lastname: Houdart
authors.forename: P H
authors.initials: PH
authors.lastname: Gaulard
mesh_terms: D000328:Adult; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D004854:Herpesvirus 4, Human / Q000235:genetics / Q000302:isolation & purification* / Q000472:pathogenicity; D006801:Humans; D016130:Immunophenotyping; D017403:In Situ Hybridization; D007414:Intestinal Neoplasms / Q000276:immunology / Q000473:pathology* / Q000821:virology*; D016399:Lymphoma, T-Cell / Q000276:immunology / Q000473:pathology* / Q000821:virology*; D008297:Male; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000235:genetics / Q000378:metabolism
chemical_list: D016692:Receptors, Antigen, T-Cell, gamma-delta