Treatment of Aggressive Mantle Cell Lymphoma

Clinical trials have not determined which therapeutic option offers the best long-term survival for patients with previously untreated mantle cell lymphoma (MCL). The situation is unclear because MCL is a relatively rare disease (4,000 new cases per year in the United States), and study evidence has accrued slowly over the past decade. A historical perspective may help to explain the state of the evidence.

MCL was first described in the 1980s as a distinct entity from small lymphocytic lymphoma/chronic lymphocytic lymphoma or follicular lymphoma. When treated with oral alkylators and infusional cytotoxic agents available at the time, MCL appeared to relapse sooner and more frequently than other indolent lymphomas. When purine analogues also proved ineffective in the 1990s, MCL was viewed as an aggressive lymphoma without a discernible cure. This view ultimately led to an aggressive treatment paradigm that incorporated all available modalities in the early 2000s: R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) followed by high-dose cytarabine with or without a platinum agent, plus autologous stem cell transplant (SCT) plus rituximab maintenance.[1,2]