Small bowel lymphoma in unrecognized coeliac disease: a cause for concern?

BACKGROUND AND AIM: It is not clear whether the increased risk of small bowel lymphoma seen in typical coeliac disease also applies to unrecognized or screening-detected coeliac patients. The aim of the present study was to determine the features of small bowel lymphoma and whether it is associated with unrecognized coeliac disease at the time of presentation.
DESIGN: A retrospective search by the five pathology laboratories serving Northern Ireland, UK.
METHODS: Snomed searches were used to identify cases of adenocarcinoma (SBA) and lymphoma (SBL) affecting the small intestine between 1987 and 1996. Pathology reports were obtained and analysed with respect to the clinical features, site of pathology, type of tumour, grading and the presence or absence of distant villous atrophy.
RESULTS: One hundred and thirty-eight cases were identified of whom 69 (44 men; mean age 60.2 years) were SBL and 69 (41 men; mean age 68.0 years) were SBA. Comparing the SBL to the SBA group the clinical presentation was perforation (10 vs 1, P= 0.009), small intestinal obstruction (20 vs 20, P= 1.0) and small intestinal mass (13 vs 15, P= 0.67). B-cell lymphomas occurred in 20 cases, T-cell lymphomas in 24 cases and 25 were unclassified. There was one known coeliac patient in the SBL group and none in the SBA group (P = 1.0). Villous atrophy at a distant site was recorded in 13 patients in the SBL group compared to none in the SBA group, all of these patients having T-cell lymphomas.
CONCLUSIONS: Similar numbers of SBL and SBA occurred in the study period. Their clinical presentation was similar although perforation was more common in the SBL group. One-third of all SBL patients (over half of T-cell lymphomas) had distant villous atrophy, indicating that small bowel lymphoma appears to be significantly associated with unrecognized coeliac disease.