Mantle cell lymphoma: state of the art.

Mantle cell lymphoma (MCL) accounts for approximately 6% of all non-Hodgkin lymphomas (NHLs). The median age at diagnosis is 60 to 70 years, although MCL may occur in younger patients. Between 75% and 80% of patients are males. MCL usually presents as stage III/IV disease, and extranodal involvement is quite common, particularly in the bone marrow, blood, and gastrointestinal tract. Until recently, MCL was considered a disease with an overall poor prognosis. With the introduction of more aggressive induction chemotherapy regimens (especially those incorporating high-dose cytarabine), the anti-CD20 monoclonal antibody rituximab, and the more widespread use of consolidation with high-dose therapy and autologous stem cell rescue, outcomes have significantly improved. Some patients have even experienced long-term remissions. New insights into the biology of MCL, most prominently the role of the B-cell receptor pathway, have shed new light on treatment approaches for this disease. In this article, we will review current therapeutic approaches for MCL, as well as experimental ones.