PRIMARY INTESTINAL GAMMA-DELTA T-CELL LYMPHOMA WITH EVIDENCE OF EPSTEIN-BARR-VIRUS

Aims: Primary intestinal T-cell lymphomas account for about 5% of all primary gastrointestinal lymphomas and are mostly associated with coel iac disease, They usually express the CD3-associated T-cell receptor a lpha/beta heterodimer and HML1, and some are related with Epstein-Barr virus (EBV), As far as we know, the present report describes the firs t case of primary gamma-delta (gamma delta) EBV-associated intestinal T-cell lymphoma without enteropathy. Only hepatosplenic, nasal and cut aneous gamma delta T-cell lymphomas have previously been described, Me thods and results: Our case concerned a 43-year-old man with no histor y of coeliac disease, who presented with multifocal small bowel involv ement showing high grade T-cell lymphoma with medium sized and large p leomorphic cells and a small pleomorphic T-cell component, Angioinvasi on 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 CD 4, CDS, CD5, or HML1, Most tumour cells were positive for the cytotoxi c granular proteins TiAl and granzyme B, Rearrangement of the TCR gamm a chain gene was demonstrated by polymerase chain reaction and in-situ hybridization with EBER probes revealed strong nuclear positivity in virtually all neoplastic cells, Conclusion: We described the first cas e of primary intestinal gamma delta T-cell lymphoma without enteropath y in which EBV might fulfil a pathogenic role.