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Blood, Vol. 94 No. 1 (July 1), 1999:
pp. 260-264
Mucosal Intra-epithelial Lymphocytes in Enteropathy-Associated
T-Cell Lymphoma, Ulcerative Jejunitis, and Refractory Celiac
Disease Constitute a Neoplastic Population
Eniko Bagdi,
Timothy C. Diss,
Philippa Munson, and
Peter G. Isaacson
From the Department of Histopathology, Royal Free and University
College Medical School, University College London, London, UK.
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.
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