Haema 2013; 4(2):171-182
by Gerassimos A. Pangalis1, Sotirios Sachanas1, Theodoros P. Vassilakopoulos2, Maria K. Angelopoulou2
1 Department of Hematology, Athens Medical Center, Psychikon Branch, Athens,
2Department of Haematology, University of Athens, Greece
Abstract
Mantle cell lymphoma (MCL) represents approximately 3–10% of all non-Hodgkin lymphomas. MCL combines the “downside” of both high and low grade NHL; an aggressive clinical course, but with a pattern of resistant and relapsing disease rendering it incurable to standard therapy. Recent improvement has been achieved by the successful introduction of monoclonal antibodies and dose-intensified approaches including autologous stem cell transplantation (ASCT) strategies. With the exception of allogeneic hematopoietic stem cell transplantation, current treatment approaches are non-curative and the corresponding survival curves are characterized by a delayed, but continuous decline and a median survival of 4 to 6 years. However, recently, a subset of long-term survivors have been identified with a rather indolent clinical course even after conventional treatment strategies only. Emerging strategies such as proteasome inhibitors, IMIDs, mTOR inhibitors and others show comparable efficacy to conventional chemotherapy in relapsed MCL.