title: Natural killer-like T-cell lymphoma of the small intestine with a distinct immunophenotype and lack of association with gluten-sensitive enteropathy.
abstract: We report the case of a large natural killer (NK)-like T-cell lymphoma that involved the ileum and displayed a distinct immunophenotype and complex karyotype. The patient exhibited no evidence of gluten-sensitive enteropathy (celiac disease) or any other type of enteropathy as determined by clinical history, endoscopy, and serology for immunoglobulin A (IgA) antiendomysial and IgG antigliadin antibodies. Molecular studies demonstrated a clonal T-cell receptor gamma chain gene rearrangement. Immunophenotype analysis showed expression of intestinal epithelium-homing receptor CD103, CD7, cytoplasmic CD3 epsilon, CD56, and CD16 but no other T- or NK-cell markers. Cytogenetic analysis of the malignant cells revealed multiple chromosomal abnormalities indicative of a biologically advanced, high-grade lymphoma. A novel subset of normal intestinal intraepithelial lymphocytes, bearing a similar phenotype, has been described; moreover, this subset diminishes, rather than expands, in gluten-sensitive enteropathy. This case supports the notion that lymphomas involving the small intestine represent a heterogeneous group of lymphomas with diverse pathogenetic mechanisms.
authors.affiliation: Department of Pathology and Laboratory Medicine, University of Pennsylvania Medical Center, Philadelphia 19104, USA.
authors.forename: Constance M
authors.initials: CM
authors.lastname: Yuan
authors.forename: Steven
authors.initials: S
authors.lastname: Stein
authors.forename: John H
authors.initials: JH
authors.lastname: Glick
authors.forename: Mariusz A
authors.initials: MA
authors.lastname: Wasik
mesh_terms: D015703:Antigens, CD / Q000032:analysis / Q000276:immunology; D015415:Biomarkers / Q000032:analysis / Q000097:blood; D014408:Biomarkers, Tumor / Q000032:analysis / Q000097:blood / Q000276:immunology; D002446:Celiac Disease / Q000097:blood / Q000175:diagnosis*; D006801:Humans; D016130:Immunophenotyping / Q000379:methods*; D007414:Intestinal Neoplasms / Q000097:blood / Q000737:chemistry / Q000175:diagnosis* / Q000235:genetics*; D007421:Intestine, Small / Q000737:chemistry / Q000473:pathology*; D007694:Killer Cells, Natural / Q000737:chemistry / Q000378:metabolism / Q000473:pathology*; D016399:Lymphoma, T-Cell / Q000097:blood / Q000737:chemistry / Q000175:diagnosis* / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D010641:Phenotype
chemical_list: D015703:Antigens, CD; D015415:Biomarkers; D014408:Biomarkers, Tumor