title: Mantle Cell Lymphoma.
abstract: Mantle cell lymphoma (MCL) is an uncommon subtype of non-Hodgkin lymphoma previously considered to have a poor prognosis. Large gains were made in the first decade of the new century when clinical trials established the importance of high-dose therapy and autologous stem-cell rescue and high-dose cytarabine in younger patients and the benefits of maintenance rituximab and bendamustine in older patients. In particular, greater depth of understanding of the molecular pathophysiology of MCL has resulted in an explosion of specifically targeted new efficacious agents. In particular, agents recently approved by the Food and Drug Administration include the proteasome inhibitor bortezomib, immunomodulator lenalidomide, and Bruton's tyrosine kinase inhibitor ibrutinib. We review recent advances in the understanding of MCL biology and outline our recommended approach to therapy, including choice of chemoimmunotherapy, the role of stem-cell transplantation, and mechanism-based targeted therapies, on the basis of a synthesis of the data from published clinical trials.
authors.affiliation: Chan Yoon Cheah, Sir Charles Gairdner Hospital and PathWest Laboratory Medicine WA, Nedlands; Chan Yoon Cheah, University of Western Australia, Crawley, Western Australia; John F. Seymour, Peter MacCallum Cancer Centre, East Melbourne; John F. Seymour, University of Melbourne, Parkville, Victoria, Australia; and Chan Yoon Cheah and Michael L. Wang, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Chan Yoon
authors.initials: CY
authors.lastname: Cheah
authors.affiliation: Chan Yoon Cheah, Sir Charles Gairdner Hospital and PathWest Laboratory Medicine WA, Nedlands; Chan Yoon Cheah, University of Western Australia, Crawley, Western Australia; John F. Seymour, Peter MacCallum Cancer Centre, East Melbourne; John F. Seymour, University of Melbourne, Parkville, Victoria, Australia; and Chan Yoon Cheah and Michael L. Wang, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: John F
authors.initials: JF
authors.lastname: Seymour
authors.affiliation: Chan Yoon Cheah, Sir Charles Gairdner Hospital and PathWest Laboratory Medicine WA, Nedlands; Chan Yoon Cheah, University of Western Australia, Crawley, Western Australia; John F. Seymour, Peter MacCallum Cancer Centre, East Melbourne; John F. Seymour, University of Melbourne, Parkville, Victoria, Australia; and Chan Yoon Cheah and Michael L. Wang, The University of Texas MD Anderson Cancer Center, Houston, TX. miwang@mdanderson.org.
authors.forename: Michael L
authors.initials: ML
authors.lastname: Wang
mesh_terms: D000225:Adenine / Q000031:analogs & derivatives; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D000069461:Bendamustine Hydrochloride / Q000008:administration & dosage; D000069286:Bortezomib / Q000494:pharmacology; D003561:Cytarabine / Q000008:administration & dosage; D004334:Drug Administration Schedule; D020869:Gene Expression Profiling; D015972:Gene Expression Regulation, Neoplastic; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D060828:Induction Chemotherapy; D000077269:Lenalidomide; D020522:Lymphoma, Mantle-Cell / Q000175:diagnosis* / Q000188:drug therapy* / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D060046:Maintenance Chemotherapy; D058990:Molecular Targeted Therapy / Q000379:methods*; D009367:Neoplasm Staging; D010880:Piperidines; D011379:Prognosis; D011720:Pyrazoles / Q000494:pharmacology; D011743:Pyrimidines / Q000494:pharmacology; D012307:Risk Factors; D000069283:Rituximab / Q000008:administration & dosage; D013792:Thalidomide / Q000031:analogs & derivatives / Q000494:pharmacology; D014182:Transplantation, Autologous
chemical_list: D000970:Antineoplastic Agents; D010880:Piperidines; D011720:Pyrazoles; D011743:Pyrimidines; D003561:Cytarabine; C551803:ibrutinib; D000069283:Rituximab; D013792:Thalidomide; D000069286:Bortezomib; D000069461:Bendamustine Hydrochloride; D000077269:Lenalidomide; D000225:Adenine