Enteropathy associated T cell lymphoma: common in coeliac disease

Abstract

A 56-year-old male admitted with haematemesis and epigastric pain and severe weight loss on a background of coeliac disease. Computed tomography (CT) abdomen revealed a thickening of the mucosal folds of a short segment of jejunum. He deteriorated and had an exploratory laparotomy and bowel resection with side-side jejojejunal stapled anastomosis and extended right hemicolectomy and ileocolic anastomosis. Histology demonstrated multifocal high-grade malignant T cell lymphoma. Coeliac disease is a very common lifelong disorder. It is associated with osteoporosis, infertility, autoimmune disorders and increased risk of malignancy including an increased risk of non-Hodgkin's lymphoma (NHL) especially of the T cell type. Enteropathy-type T cell lymphoma is associated with a very poor prognosis. There is significant evidence that adherence to a gluten-free diet decreases the risk of developing enteropathy-type T cell lymphoma and helps to prevent development of autoimmune diseases, diabetes mellitus and osteoporosis in patients with coeliac disease.

Figure 1

CT image.

Figure 2

H&E: sheets of large tumour cells with prominent nucleoli and numerous mitoses.

Figure 3

Low-power view. H&E section shows flat mucosal surface (total villous atrophy), submucosal oedema and serosal tumour.

Figure 4

Total to subtotal villous atrophy with increased intraepithelial lymphocytes.

Figure 5

CD3 (T cell marker) demonstrating intraepithelial lymphocytes.