Mantle cell lymphoma: therapeutic strategies are different from CLL.

In contrast to the typical course of chronic lymphocytic lymphoma and despite an indolent lymphoma-like presentation, the clinical outcome of mantle cell lymphoma (MCL) is dismal, with a median survival time of 3 years and virtually no long-term survivors. Most patients are diagnosed with advanced stage III/IV disease. Although clinical studies did not prove a clear superiority of anthracyclin-containing combinations, CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone)-like regimens represent the standard therapeutic approach in MCL. Recent randomized studies have shown a benefit of a combined immunochemotherapy strategy (chemotherapy plus rituximab) increasing the complete and overall response rates, whereas further follow-up is pending for evaluation of the progression-free and overall survival. In patients younger than 65 years, a dose-intensive consolidation comprising high-dose radiochemotherapy and subsequent autologous stem cell transplantation after a CHOP-like induction results in an improved progression-free survival. However, despite the benefits of this multimodal approach, most patients relapse even after high-dose therapy. The only curative approach is allogeneic stem cell transplantation, which may be adapted to the elderly MCL patient cohort by modified dose-reduced conditioning regimens. Prospective randomized trials remain critical to further improve the clinical course of MCL with the addition of newer treatment modalities, such as radioactively labeled antibodies and targeted therapies (eg, flavopiridol and PS-341).