Coeliac disease and lymphoma

Abstract

The incidence of malignancy is approximately twofold greater in coeliac disease than in the general population. Lymphomas of the small intestine, often multifocal and diffuse, were found in 0-18% of coeliac patients, representing about 50% of all malignancies. Most of lymphomas are of T-cell origin. Enteropathy-associated T-cell lymphomas (EATL) usually occur in older patients with long-lasting disease who do not strictly adhere to a gluten-free diet. It was shown that an appropriate diet, early instituted and rigorously followed, could prevent the development of lymphomas, supporting the view that coeliac disease is a risk factor for lymphoma. In rare cases, lymphomas can be diagnosed concurrently or at short time after coeliac disease, and debate continues as to whether adult-onset coeliac disease is itself a form of lowgrade lymphoma, at least in some patients. Controversy has recently emerged regarding the magnitude of lymphoma as a complication of coeliac disease. The previous studies, necessarily limited to symptomatic coeliac patients, might have lead to biased estimates of risk. A large Italian multicentre study in patients with non-Hodgkin lymphoma indicates that the risk to develop lymphoma in coeliac disease, although greater than in the general population, is lower than previously estimated.