Mantle Cell Lymphoma-Lymphomatous Intestinal Polyposis

Abstract

Mantle Cell Lymphoma (MCL) is a rare and aggressive form of the mature B cell non-Hodgkin lymphomas (NHL) that commonly affects older individuals and has one of the worst outcomes of all the lymphomas. It comprises of about 7% of adult NHLs in the U.S. and Europe with an incidence of 4 to 8 cases per million persons per year. Most present with lymphadenopathy, extranodal disease is infrequent with GI tract involvement referred to as lymphomatous intestinal polyposis. Patients may have systemic B symptoms, such as fever, night sweats, and weight loss. Diagnosis often requires a combination of tissue biopsy, immunohistochemistry, karyotyping or fluorescence in situ hybridization (FISH). Prognosis is dismal especially with blastoid variant. It was first described and also reviewed by Cornes in 1961. Treatment may alter progression that consists of combination chemotherapy plus immunotherapy with or without autologous hematopoietic cell transplantation (HCT). Surgery and radiation is usually of no benefit except in cases of bowel obstruction or palliation. 76 y/o M with PMHx of Barrett's esophagus, GERD, Anemia, and colon polyp presents for surveillance of his colon polyp and Barrett's esophagus. He denies any dysphagia, abdominal pain, n/v/d, decrease appetite, GI bleed, fever, night sweats, or weight loss. Physical examination was normal. Labs were unremarkable except mild anemia. A scheduled surveillance EGD and colonoscopy showed negative Barrett's but revealed numerous polypoid lesions throughout left and right colon all pathologically confirming MCL. Immunochemistries were positive for CD5, Bcl-2, CD20, and PAX-5 with strong expression of Cyclin D-1. CD-3 was also positive. CD23 stain and PCR for t(11;14) were pending. Oncology referral was made for treatments of MCL. Mantle cell lymphoma with GI involvement i.e. lymphomatous intestinal polyposis is a rare phenomenon worth sharing for future research. The difficulty lies in differentiating disease burden because those with GI involvement are likely to have worse outcome. We conclude that we need a better predictor for highrisk population with diagnosis of MCL. We propose a retrospective study of all MCL cases to find a possible common factor for early recognition. Early treatment may improve survival, prevent recurrences, and potentially decrease resistance to treatment, especially in the blastoid variant of MCL.