title: Mantle cell lymphoma: evolving management strategies.
abstract: Mantle cell lymphoma (MCL) is a rare and aggressive form of non-Hodgkin's lymphoma that generally affects older individuals and continues to have one of the worst outcomes of all the lymphomas. Over the last decade, there has been a widespread adoption of cytarabine-based therapy in younger patients, and the incorporation of rituximab into chemotherapeutic regimens has become an evidence-based standard of care. However MCL remains a largely incurable disease, and following relapse, it can be a challenge to manage. Although it is possible to define prognosis reliably, there are, as yet, no clear diagnostic or response-adjusted parameters that can help to guide therapeutic decisions. However, there are a number of highly active targeted therapies that are moving into the clinic that are set to transform the therapeutic paradigm for this disease in the very near future. This review will explore the molecular pathogenesis of MCL and the current and evolving therapeutic strategies for this disease.
authors.affiliation: Hematopathology Section, Department of Pathology, Hospital Clinic, Institut d'Investigacions Biomèdiques August Pi i Sunyer, University of Barcelona, Barcelona, Spain; and.
authors.forename: Elias
authors.initials: E
authors.lastname: Campo
authors.affiliation: Plymouth University Peninsula Schools of Medicine and Dentistry and Derriford Hospital, Plymouth, United Kingdom.
authors.forename: Simon
authors.initials: S
authors.lastname: Rule
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001897:Boronic Acids / Q000627:therapeutic use; D000069286:Bortezomib; D002986:Clinical Trials as Topic; D003561:Cytarabine / Q000627:therapeutic use; D006801:Humans; D020522:Lymphoma, Mantle-Cell / Q000235:genetics / Q000628:therapy*; D008495:Medical Oncology / Q000379:methods; D009154:Mutation; D049268:Positron-Emission Tomography; D011379:Prognosis; D011719:Pyrazines / Q000627:therapeutic use; D000069283:Rituximab; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D001897:Boronic Acids; D011719:Pyrazines; D003561:Cytarabine; D000069283:Rituximab; D000069286:Bortezomib