Mucosal intra-epithelial lymphocytes in enteropathy-associated T-cell lymphoma, ulcerative jejunitis, and refractory celiac disease constitute a neoplastic population.
Loss of response to a gluten-free diet (refractory sprue) and ulcerative jejunitis are complications of celiac disease that may progress to enteropathy-associated T-cell lymphoma (EATL). Both conditions are characterized by the presence of a nonlymphomatous monoclonal T-cell population in the enteropathic mucosa. In EATL, a similar monoclonal population that shows clonal identity with the lymphoma itself is also present in the enteropathic mucosa. In this study we show that in all three circumstances the monoclonal T-cell population is constituted by cytologically normal, noninvasive intraepithelial T lymphocytes that share an identical aberrant immunophenotype with EATL. Patients with refractory sprue and/or ulcerative jejunitis are, therefore, suffering from a neoplastic T-cell disorder for which hematological treatment strategies need to be devised.
Bagdi
E
E
Department of Histopathology, Royal Free and University College Medical School, University College London, London, UK.
Diss
T C
TC
Munson
P
P
Isaacson
P G
PG
D002446:Celiac Disease / Q000276:immunology / Q000473:pathology*; D004386:Duodenum / Q000276:immunology / Q000473:pathology; D004751:Enteritis / Q000276:immunology / Q000473:pathology; D006801:Humans; D007413:Intestinal Mucosa / Q000276:immunology* / Q000473:pathology*; D007579:Jejunal Diseases / Q000276:immunology / Q000473:pathology*; D018442:Lymphoma, B-Cell, Marginal Zone / Q000276:immunology / Q000473:pathology*; D016399:Lymphoma, T-Cell / Q000276:immunology / Q000473:pathology*; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology*