PuSH - Publication Server of Helmholtz Zentrum München

Dreyling, M.* ; Kluin-Nelemans, H.C.* ; Beà, S.* ; Hartmann, E.* ; Salaverria, I.* ; Hutter, G. ; Perez-Galan, P.* ; Roue, G.* ; Pott, C.* ; Le Gouill, S.* ; Cortelazzo, S.* ; Rule, S.* ; Hess, G.* ; Zaja, F.* ; Vitolo, U.* ; Szymczyk, M.* ; Walewski, J.* ; Ribrag, V.* ; Unterhalt, M.* ; Hermine, O.* ; Hoster, E* ; European MCL Network (*)

Update on the molecular pathogenesis and clinical treatment of mantle cell lymphoma: Report of the 10th annual conference of the European Mantle Cell Lymphoma Network.

Leuk. Lymphoma 52, 2226-2236 (2011)
DOI PMC
Open Access Green as soon as Postprint is submitted to ZB.
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 shows an aggressive clinical course with a continuous relapse pattern and a median survival of only 3-5 years. However, recently a subset of up to 15% long-term survivors has been identified with a rather indolent clinical course. Advanced stage disease is usually apparent already at first clinical manifestation; in general, conventional chemotherapy is only palliative and 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 Ara-C to a rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP)-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 control of cell cycle machinery and impairment of several apoptotic pathways. Combination strategies are currently being investigated in numerous trials, but their introduction into clinical practice and current treatment algorithms remains a challenge. Future strategies will apply individualized approaches according to the molecular risk profile of the patient. At the annual conference in Warsaw, recent results of molecular pathogenesis, analyses of current clinical trials and new study concepts were discussed.
Altmetric
Additional Metrics?
[➜Log in]
Edit extra informations Login
Publication type Article: Journal article
Document type Review
Corresponding Author
Keywords Mantle cell lymphoma; clinical treatment; NON-HODGKINS-LYMPHOMA; BENDAMUSTINE PLUS RITUXIMAB; PROGRESSION-FREE SURVIVAL; GENE-EXPRESSION; B-CELL; MCL NETWORK; IMMUNOCHEMOTHERAPY; TRANSLOCATIONS; AMPLIFICATION; MULTICENTER
ISSN (print) / ISBN 1042-8194
e-ISSN 1029-2403
Journal Leukemia and Lymphoma
Quellenangaben Volume: 52, Issue: 12, Pages: 2226-2236 Article Number: , Supplement: ,
Publisher Informa Healthcare
Publishing Place London
Non-patent literature Publications
Reviewing status Peer reviewed
Institute(s) CCG Pathogenesis of Acute Myeloid Leukemia (KKG-KPL)