Type II Enteropathy-associated T-cell lymphoma: report of a Tunisian case

S. Mejri, H. Nfoussi, S. Attafi, B. Chelly, A. Zehani, I. Chelly, H. Azzouz, W. Rekik, R. Rhaiem, W. Rebaï, Z. Bensafta, S. Haouet, N. Kchir

Abstract


Enteropathy-associated T-cell lymphoma (EATL) is a rare gastrointestinal non-Hodgkin’s lymphoma originating from intraepithelial T lymphocyte. There are two types of EATL: Type I which is associated with celiac disease and Type II (10-20% of cases) also referred to as monomorphic CD56+ intestinal T-cell lymphoma. The type II EATL is not associated with celiac disease although the mucosa adjacent to the invasive tumour may show enteropathy- like changes.We propose to study the clinic-pathological features of this entity with a focus on its outcome.
We report the case of a 61 year-old woman presented with persistent diarrhea and abdominal pain lasting since 3 months. Her abdominal CT showed a uniform circumferential and segmental wall thickening of the ileum . The patient had a diagnostic laparoscopy .
The laparoscopy showed an ileal tumor measuring 8cm, perforated and clogged by the sigmoid mesocolon with several circumferential lesions. A resection of 20cm of the small intestine was done. Histologic findings showed tumor cells diffusely infiltrating the whole intestinal mucosa, involving the resection margin of the colon. The ileal resection margin didn’t show tumor infiltration. The lymphoma cells were small to medium-sized, with round, hyperchromatic nuclei, having a stippled chromatin pattern. The adjacent intestinal mucosa didn’t show villous atrophy. Immunohistochemical staining of the tumor cells expressed CD56,CD3,CD8 and CD4 but they were negative for CD20.The final diagnosis confirmed monomorphic CD56+ intestinal T-cell lymphoma not associated with enteropathy. The patient died 36days after surgery.
The type II EATL is an uncommon intestinal lymphoma with very poor prognosis and high mortality rate.

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