• Christian Geisler, Arne Kolstad, Anna Laurell, Riikka Räty, Nordic Lymphoma Group, and Mantle Cell Lymphoma Subcommittee.
• Department of Hematology, Rigshospitalet, Copenhagen, Denmark. christian.geisler@rh.regionh.dk
• Leuk. Lymphoma. 2009 Aug 1; 50 (8): 1249-56.

AbstractMCL is a rare entity of non-Hodgkin lymphoma, hitherto considered incurable. There is no standard therapy, but the current treatment results do seem to have led to a prolongation of the median survival from 3 to 5 years. Following CHOP-like induction, high-dose radiochemotherapy, and autologous stem cell transplantation (ASCT) chemotherapy has been shown in a controlled trial to be superior in younger patients, but does not, however, lead to long-term freedom from disease. Results of recent prospective but uncontrolled trials of more intensive frontline immunochemotherapy containing cytarabine and rituximab followed by ASCT, however, now for the first time indicate plateaus of the curves of event-free, progression-free and overall survival, suggesting cure, but more studies and longer follow-up is needed. Following relapse, autologous stem-cell transplantation does not seem to be of value, but graft-versus-lymphoma effect has been documented, and allogeneic stem cell transplantation with reduced-intensity conditioning is emerging as the treatment of choice in this setting.

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