Small intestinal CD4+ T-cell lymphoma: a rare distinctive clinicopathological entity associated with prolonged survival

Abstract

Dear Editor, 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” [5]. 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. [1] 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. In January 2004, a 23-year-old man was admitted in our hospital with a 4-month history of diarrhoea, weight loss (−4 kg) and massive abdominal distension. Laboratory findings revealed marked eosinophilia (absolute eosinophil count, 6,185 per mm) and polyclonal hypergammaglobulinemia (16.7 g/l, N<16 g/l) with normal IgM, IgA and IgE levels. An abdominal computed tomography (CT) scan revealed multiple enlarged mesenteric and retroperitoneal lymph nodes. Histological examination of the ileal biopsies showed an abundant and diffuse pleomorphic infiltrate, predominantly located in the lamina propria. This infiltrate was composed mainly of smallto medium-sized cells, sometimes cleaved, with inconspicuous nucleoli and plasma cells (Fig. 1a). Crypt architecture was preserved. The villi were normal or slightly shortened. There was no increase in IELs. There was no lymphoepithelial lesion. Numerous polymorphonuclear eosinophils and extensive epithelioid granulomatous reaction were associated with the lymphoid infiltrate (Fig. 1b). Gastric and colonic biopsies were normal. A few days later, the patient developed sub-acute small bowel obstruction, and a laparoscopic ileal resection was performed (Fig. 1c). Mesenteric and ileal lymph nodes were Virchows Arch (2007) 451:1091–1093 DOI 10.1007/s00428-007-0475-7