Lymphoproliferative and intestinal malignancies in 214 patients with biopsy-defined celiac disease

Prior studies have suggested that the incidence of some neoplastic disorders, particularly malignant lymphoma, is increased in celiac disease. In the present study, lymphoproliferative and intestinal cancers in 214 consecutive biopsy-defined celiac disease patients, including 148 females (69.2%) and 66 males (30.8%), seen by a single clinician over more than 20 years were tabulated. Of the 214 patients, 151 were diagnosed with celiac disease before age 60 and 63 at or after age 60. In total, 18 malignant lymphomas and 3 small intestinal adenocarcinomas were detected. While the overall incidence of malignant lymphoma was 8.4%, similar to other European centers, the incidence in elderly celiacs in this study was 22.2%. Celiac disease was detected before or even after the diagnoses of lymphoma or small intestinal adenocarcinoma were established. In some, epithelial lymphocytosis was evident in gastric, colonic, or biliary ductal epithelium. In addition, other immune-mediated disorders, dermatitis herpetiformis, and autoimmune thyroid disease were common, suggesting a distinct clinical and pathologic phenotype in celiac disease that may predispose to malignant complications. Finally, except for a single hypopharyngeal carcinoma in a celiac disease patient with a malignant lymphoma, other malignant disorders of esophagus, stomach, and colon were not detected.