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Leukemia (2014) 28, 21172130& 2014 Macmillan Publishers Limited All rights reserved 0887-6924/14
http://www.nature.com/leu
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HOW TO MANAGE...
How to manage mantle cell lymphoma
M Dreyling1,5, S Ferrero2,5 and O Hermine3,4
Mantle cell lymphoma (MCL) is no longer a hopeless disease. Considered to carry a uniformly dismal prognosis so far, during the last years it has been rediscovered as a heterogeneous clinical and biological entity. Such a complexity has been highlighted by molecular genetics, unraveling different pathways of cell survival and progression. Concurrently, the application of new therapeutic paradigms including rituximab, high-dose cytarabine and stem cell transplantation dramatically improved treatment activity and the introduction of innovative targeted molecules has already led to new patient perspectives. In this completely new and continually evolving landscape, the clinical hemato-oncologist might feel disoriented on what are the best current strategies to handle such a critical disease and the gold standard therapeutic options for MCL. Here we address some burning questions on how to manage MCL patients, spacing from prognostic issues to the dilemma of personalized treatment in different scenarios of the disease: how to diagnose an MCL? Which are the fundamental staging procedures? What are the most reliable prognosticators? Is there a place for watch and wait? Which are the best treatment options for younger, elderly and frail patients? Which patients are addressable to high-dose therapy? What is the role of allogeneic transplantation? What is the most appropriate approach for relapsing disease in different categories of patients? What novelties are going to be introduced in the near future? The practical algorithms here discussed represent an evidence-based approach derived from results of multicenter and randomized trials.
Leukemia (2014) 28, 21172130; doi:http://dx.doi.org/10.1038/leu.2014.171
Web End =10.1038/leu.2014.171
INTRODUCTIONMantle cell lymphoma (MCL) is a distinct histological subtype occurring in both elderly (465 years) and young (o65 years)
patients, with a pathognomonic chromosomal translocation t(11;14).1 During the last three decades MCL was considered as a disease with a uniformly dismal prognosis; however, with the introduction of high-dose cytarabine chemotherapy ( auto-logous stem cell transplantation, SCT) and anti-CD20 antibody therapy with rituximab especially the outcome of younger patients has improved signicantly, with some patients experiencing long-term disease-free survival.27 At the same
time, thanks to the promising results of combined induction conventional chemotherapy and rituximab, followed...