Mantle cell lymphoma.

Mantle cell lymphoma is an aggressive non-Hodgkin's lymphoma that remains incurable with current chemotherapeutic approaches. Despite response rates to many regimens of 50% to 70%, the disease typically progresses after chemotherapy with a median survival time of approximately 3 years. There is no clear standard approach for treating mantle cell lymphoma, making it critical that appropriate patients be enrolled in clinical trials. Off protocol, chemotherapy with chlorambucil, CVP (cyclophosphamide, vincristine, and prednisone), or CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) can be used in patients who are not candidates for aggressive therapy. Intensive combination chemotherapy regimens have high response rates and may prolong time to progressive disease. High-dose therapy with autologous stem cell transplantation may, but does not appear to, provide longer time to progression, although it may improve survival. For young patients with matched donors, allogeneic transplant is promising in the limited numbers of patients treated. Other agents, including rituximab, fludarabine, and cladribine, have demonstrated activity, but these agents do not appear to offer survival advantages over combination chemotherapy.