Mantle Cell Lymphoma: Biology, Clinical Presentation, and Therapeutic Approaches

Martin Dreyling
2014 American Society of Clinical Oncology Educational Book  
on behalf of the European MCL Network OVERVIEW Mantle cell lymphoma is molecularly characterized by the chromosomal translocation t(11;14) (q13;q32) that results in a constitutional overexpression of the cell cycle regulator protein cyclin D1. Generally, the disease is characterized by rapid relapses and poor long-term outcome. However, a subset of patients with indolent disease has been identified. Randomized trials have demonstrated the superiority of dose intensified, cytarabine-containing
more » ... duction with or without autologous stem cell transplantation in younger patients. In elderly patients, a rituximab-based maintenance has significantly prolonged progression-free and overall survival after treatment with rituximab, cyclophosphamide, doxorubicin hydrochloride, vincristine, and prednisone (R-CHOP). Unfortunately, the vast majority of patients will eventually relapse. Numerous molecular targeting strategies (bortezomib, lenalidomide, temsirolimus, and especially inhibitors of the B-cell receptor pathway) have achieved high response rates in phase II studies and should be strongly considered in relapsed disease.
doi:10.14694/edbook_am.2014.34.191 pmid:24857076 fatcat:wkih3czy6ngpzh6o5uvqf3hcmi