Mantle Cell Lymphoma

Abstract

Mantle cell lymphoma (MCL) is a B-cell neoplasm thought to derive from mantle zone B-cells and characterized by translocations involving the CCND1 gene, most commonly t(11;14)(q13;q23). The classical variant of MCL is comprised of small- to medium-sized cells with irregular nuclear contours. Aggressive variants of MCL include tumors composed of lymphoblast-like (blastoid) or pleomorphic cells.

MCL represents approximately 6 % of all non-Hodgkin lymphomas. The median age of MCL patients is 60 years, and the disease exhibits a male predilection with a male to female ratio of approximately 3–1. B symptoms occur in 30–40 % of patients, and 70–80 % of patients present with stage III or IV disease. Relatively low levels of lymphocytosis probably do not influence survival significantly, but overt mantle cell leukemia has been associated with a worse prognosis. Subclinical extranodal disease is common, particularly involving the gastrointestinal tract, but overt extranodal disease is less common and usually occurs in association with nodal involvement. A small subset of patients presents with symptoms related to prominent involvement of the gastrointestinal tract by numerous polyps that extend from the gastroesophageal junction to anorectal junction (multiple lymphomatous polyposis).