Current Approaches to Mantle Cell Lymphoma: Diagnosis, Prognosis, and Therapies

Mantle cell lymphoma (MCL) is a unique lymphoma subtype, both biologically and clinically. Virtually all cases are characterized by a common genetic lesion, t(11;14), resulting in overexpression of cyclin D1. The clinical course is moderately aggressive, and the disease is considered incurable. Considerable biologic and clinical heterogeneity exists, with some patients experiencing a rapidly progressive course, while others have disease that is readily managed. New tools exist for risk stratification and may allow for a more personalized approach in the future. Landmark studies have been completed in recent years and outcomes appear to be improving. Randomized clinical trials have clarified the role of high-dose cytarabine (Ara-C) for younger patients and have demonstrated a role for maintenance rituximab therapy. Multiple areas of uncertainty remain, however, and are the focus of ongoing research. This review focuses on (1) strategies to differentiate between aggressive and less aggressive cases, (2) understanding who should receive hematopoietic stem cell transplantation, and (3) the role for maintenance therapy in MCL.