title: Update on the molecular pathogenesis and clinical treatment of mantle cell lymphoma: report of the 11th annual conference of the European Mantle Cell Lymphoma Network.
abstract: Mantle cell lymphoma (MCL) is a distinct subtype of malignant lymphoma characterized by the chromosomal translocation t(11;14)(q13;q32), resulting in constitutional overexpression of cyclin D1 and cell cycle dysregulation in virtually all cases. Clinically, MCL displays an aggressive course, with a continuous relapse pattern and a median survival of only 3-7 years. However, a subset of up to 15% long-term survivors has recently been identified with a rather indolent clinical course. In general, conventional chemotherapy is only palliative and the median duration of remissions is only 1-2 years. In 2000, the European MCL Network (http://www.european-mcl.net) was founded, which consists of 15 national lymphoma study groups supplemented by experts in hematopathology, cytogenetics and molecular genetics. During the last decade, the European consortium has successfully initiated the largest phase III trials in MCL worldwide. In the current study generation, the addition of high dose cytosine arabinoside (Ara-C) to an R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone)-like regimen followed by myeloablative consolidation achieved a significant improvement of progression-free survival. Similarly, in elderly patients, rituximab maintenance until progression led to a marked prolongation of remission duration. Emerging strategies include proteasome inhibitors, immune modulatory drugs (IMiDs), mammalian target of rapamycin (mTOR) inhibitors and others, all based on the dysregulated cell cycle machinery and impairment of several signaling transduction and apoptotic pathways. Future strategies will apply individualized approaches according to the molecular risk profile of the patient. At the annual conference in Lisbon, recent results of molecular pathogenesis, analyses of current clinical trials and new study concepts were discussed.
authors.affiliation: Department of Medicine III, University Hospital Großhadern/LMU Munich, Germany. Martin.Dreyling@med.uni-muenchen.de
authors.forename: Martin
authors.initials: M
authors.lastname: Dreyling
authors.forename: Hanneke C
authors.initials: HC
authors.lastname: Kluin-Nelemans
authors.forename: Sílvia
authors.initials: S
authors.lastname: Beà
authors.forename: Wolfram
authors.initials: W
authors.lastname: Klapper
authors.forename: Niclas
authors.initials: N
authors.lastname: Vogt
authors.forename: Marie-Helene
authors.initials: MH
authors.lastname: Delfau-Larue
authors.forename: Grit
authors.initials: G
authors.lastname: Hutter
authors.forename: Chan
authors.initials: C
authors.lastname: Cheah
authors.forename: Annalisa
authors.initials: A
authors.lastname: Chiappella
authors.forename: Sergio
authors.initials: S
authors.lastname: Cortelazzo
authors.forename: Christiane
authors.initials: C
authors.lastname: Pott
authors.forename: Georg
authors.initials: G
authors.lastname: Hess
authors.forename: Carlo
authors.initials: C
authors.lastname: Visco
authors.forename: Umberto
authors.initials: U
authors.lastname: Vitolo
authors.forename: Pavel
authors.initials: P
authors.lastname: Klener
authors.forename: Igor
authors.initials: I
authors.lastname: Aurer
authors.forename: Michael
authors.initials: M
authors.lastname: Unterhalt
authors.forename: Vincent
authors.initials: V
authors.lastname: Ribrag
authors.forename: Eva
authors.initials: E
authors.lastname: Hoster
authors.forename: Olivier
authors.initials: O
authors.lastname: Hermine
mesh_terms: D000367:Age Factors; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002986:Clinical Trials as Topic; D006801:Humans; D020522:Lymphoma, Mantle-Cell / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy*; D013792:Thalidomide / Q000627:therapeutic use
chemical_list: D000970:Antineoplastic Agents; D013792:Thalidomide