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Primary intestinal gamma‐delta T‐cell lymphoma with evidence of Epstein–Barr virus
Author(s) -
A Lavergne,
Isabelle Brochériou,
Marie-Hélène Delfau,
Christiane CopieBergman,
R Houdart,
Philippe Gaulard
Publication year - 1998
Publication title -
histopathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.626
H-Index - 124
eISSN - 1365-2559
pISSN - 0309-0167
DOI - 10.1046/j.1365-2559.1998.00394.x
Subject(s) - cd5 , lymphoma , t cell lymphoma , epstein–barr virus , t cell receptor , gene rearrangement , cd8 , pathology , cd3 , biology , t cell , granzyme b , virus , immunology , medicine , antigen , immune system , gene , biochemistry
Aims: 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 α/β 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 (γδ) EBV‐associated intestinal T‐cell lymphoma without enteropathy. Only hepatosplenic, nasal and cutaneous γδ T‐cell lymphomas have previously been described. Methods and 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 γδ phenotype (CD3+, CD2+, TCRδ1+, Vδ2+ and βF1–) 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 TCRγ 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 case of primary intestinal γδ T‐cell lymphoma without enteropathy in which EBV might fulfil a pathogenic role.