Risk of Non-Hodgkin Lymphoma in Celiac Disease

Abstract

The association between celiac disease (CD) and lymphoma has long been established. In 1937, Fairly and Mackie¹ reported the association of malignant lymphoma of the small intestine with steatorrhea. In 1962, Gough et al² described 5 cases of small intestinal lymphoma in patients with long-standing CD. A series of celiac-associated lymphomas have since been reported.^3-5 The most frequent malignancy associated with CD is a high-grade, T-cell non-Hodgkin lymphoma (NHL) of the upper small intestine, currently defined as enteropathy-associated T-cell lymphoma (EATL), that peaks in the sixth or seventh decade of life.⁶ Also CD may be associated with other NHL types of both the B- and T-cell type in either the gut or other primary sites.^7,8 Holmes et al provided evidence that dietary compliance to a gluten-free diet (GFD) reduces the risk of lymphoma and other malignancies in CD.⁹