Small intestinal CD4+ T-cell lymphoma: a rare distinctive clinicopathological entity associated with prolonged survival
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Intestinal T-cell lymphomas are rare and heterogeneous. Most of them are localised high-grade large cell lymphomas and carry a poor prognosis. The WHO classification only recognises one distinct entity of intestinal T-cell lymphoma, called “enteropathy-associated T-cell lymphoma” . This lymphoma arises in pre-existing celiac disease. The neoplastic cells infiltrate the overlying epithelium, mimicking normal intraepithelial lymphocytes (IELS). They are CD3+, CD5−, CD7+, CD8−/+ and also express the homing receptor CD103 antigen, typically seen in normal and malignant intraepithelial T lymphocytes. CD4 is always negative.
In 1999, Carbonnel et al.  reported four patients with clinico-pathological features suggesting a new distinct entity defining extensive small intestinal CD4+ T-cell lymphoma.
We had the opportunity to diagnose an additional case of this T-cell low grade lymphoma in a patient without any evidence of celiac disease, associated with prolonged survival.
KeywordsCeliac Disease Granulomatous Reaction Ileal Resection Cutaneous Lymphoma Polyclonal Hypergammaglobulinemia
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