Peritonitis as the primary manifestation of atypical T-cell lymphoma of the small bowel with unsuspected celiac disease: MDCT features

We report one case of jejunal free perforation as the initial manifestation of small bowel lymphoma in a 46 year-old man with unsuspected celiac disease. Perforation was the revealing feature of the lymphoma and led to recognition of latent, long standing celiac disease. Multidetector row computed tomography (MDCT) showed pneumoperitoneum, fluid effusion and findings suggestive for celiac disease including diffuse small bowel thickening, paucity of jejunal folds, enlarged mesenteric lymph nodes and focal jejunal mass. Histopathological analysis confirmed perforation of jejunal pleomorphic large cell, enteropathy-associated T-cell lymphoma (EATL) with marked changes of jejunal mucosa consistent with long standing celiac disease. Immunohistochemical staining on jejunal lesion was positive for CD3, CD8, and CD30 but negative for CD56 and CD57. Atrophic mucosa did not contain clonal expansion of an aberrant intraepithelial lymphocyte population. A favorable outcome was observed after emergency surgery, chemotherapy, and gluten-free diet and the patient was still alive 46 months after the initial symptoms. This case teaches us that free perforation of EATL in celiac disease may have a favorable outcome, EATL may develop in long standing celiac disease in the absence of clonal expansion of an aberrant intraepithelial lymphocyte population, and that MDCT may suggest the diagnosis preoperatively.