Monomorphic epitheliotropic T-cell lymphoma of the small intestine.

Abstract

A 64-year-old man, without celiac disease or comorbidities, presented with epigastric pain, vomiting, and unintentional weight loss. His CT scans revealed a diffuse distension of the small intestine, asymmetric thickening of the proximal jejunum without complete obstruction, and a lung mass (Figures 1a, 2 and 3). On jejunoscopy, the mucosa showed diffuse involvement with shortened villi and denuded mucosa, reduction of pleats, and atrophy, shallow ulcers, reddish plaques, enlargement of crypts, and villous edema interspersed with normal mucosa (Figures 1b and 4). On histological staining with hematoxylin–eosin, enteric mucosa with irregular trophism, crypt hypotrophy, and areas with a hyperplastic appearance and 40% of intraepithelial lymphocytes were observed (Figure 1c). Samples were also obtained from the colon, which had ulcers, and from the lung mass; and based on the immunohistochemical study of all samples, a diagnosis of metastatic monomorphic epitheliotropic intestinal T-cell lymphoma was made. The patient underwent a chemotherapy session but died after an episode of febrile neutropenia. (a) CT scan with the asymmetric thickening of the proximal jejunum without stenosis. (b) Jejunoscopy image: jejunum with crypt enlargement, hyperplastic appearance, and villous edema. (c) Histologically stained slides with hematoxylin–eosin with a dense monomorphic lymphocytic infiltrate. (d) Immunohistochemical slides with CD8+. Coronal CT with the lung mass. Cross-sectional CT with the lung mass. Junoscopy with shallow ulcers, reddish plaques, enlargement of crypts, and villous edema interspersed with normal mucosa. Monomorphic epitheliotropic intestinal T-cell lymphoma is a primary intestinal mature T-cell lymphoma caused by malignant proliferation of intraepithelial lymphocytes, representing less than 5% of all gastrointestinal lymphomas.1 It has an increased incidence in Asian and Hispanic populations and because of its poor association with celiac disease, it is nowadays classified as a different entity from enteropathy-associated T-cell lymphoma.1-3 The endoscopic presentation varies, including ulcers, reddish plaques, and atrophy; and histologically, a dense and monotonous epitheliotropic lymphocytic infiltrate immunopositive for CD3, CD8, and CD561, 4 (Figure 1d). It has a high mortality rate due to its treatment—chemotherapy followed by autologous stem cell transplantation—and intestinal complications—perforation and bleeding.5 This article has no specific funding. All authors have no conflicts of interest. Approval was obtained from the local ethics committee with the number 64877822.5.0000.0096. Approval was obtained from the local ethics committee with the number 64877822.5.0000.0096. This article has no material from other sources. Approval was obtained from the local ethics committee with the number 64877822.5.0000.0096. The data underlying this article are available in the article.