Lymphoma of the small intestine in adult coeliac disease.

Five out of 74 patients with adult coeliac disease (ACD) diagnosed from 1965 through 1977 developed intestinal lymphoma. The age at diagnosis of ACD was 54-63 years (mean 58) and in all patients an initial improvement was noted on a gluten-free diet. The interval between the diagnoses of ACD and lymphoma was 0.5-6 years (mean 3). In 4 patients the diagnosis of lymphoma was preceded by relapse of diarrhoea and loss of weight despite an adequate diet; 2 patients developed perforation of the bowel. In all patients who subsequently developed lymphoma a pronounced lymphocytopenia, 0.4-1.3 X 10(9)/l, was found at the diagnosis of ACD. It is suggested that ACD patients over 40 who have relapse of malabsorption symptoms despite an adequate diet should be investigated for the presence of an intestinal lymphoma. The suspicion of a malignant transformation may be especially strong if a previous, pronounced lymphocytopenia has been recorded. Three patients died 1-12 months after the diagnosis of lymphoma. However, 2 patients are in good condition 14 and 20 months, respectively, after laparotomy and removal of the tumour, indicating that an early diagnosis and treatment may have a reasonably palliative effect in some cases.