Primary Enteropathy-Associated T-Cell Lymphoma Type 2: An Emerging Entity?

Intestinal T-cell lymphoma is a rare hematological malignancy that can present as primary intestinal lymphoma or as a manifestation of systemic disease. Primary involvement accounts for approximately 0.1% to 0.5% of all colorectal neoplasms. It is an aggressive disease with a poor prognosis and low survival rate. Inflammatory bowel disease, celiac disease, immunosuppression, and infectious etiologies, such as Epstein-Barr and human T-lymphotropic viruses, have been reported as risk factors, but no direct causal link has been established. Herein, we examine the case of a Hispanic man 69 years of age diagnosed with positive CD3, CD7, CD8, CD43, and Bcl-2 diffuse primary colorectal T-cell lymphoma. The patient did not exhibit a concomitant autoimmune or genetic disease. Because of the patient's history of polyps, surveillance colonoscopy was performed and the diagnosis was confirmed.