title: Mantle cell lymphoma: biology, clinical presentation, and therapeutic approaches.
abstract: 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 induction 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.
authors.affiliation: From the Department of Medicine III, University Hospital Großhadern/LMU München, Munich, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Dreyling
mesh_terms: D000367:Age Factors; D000368:Aged; D000818:Animals; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use*; D014408:Biomarkers, Tumor / Q000235:genetics; D017024:Chemotherapy, Adjuvant; D002880:Chromosomes, Human, Pair 11; D002883:Chromosomes, Human, Pair 14; D018450:Disease Progression; D018572:Disease-Free Survival; D020022:Genetic Predisposition to Disease; D006801:Humans; D020522:Lymphoma, Mantle-Cell / Q000188:drug therapy* / Q000235:genetics / Q000401:mortality; D008875:Middle Aged; D058990:Molecular Targeted Therapy; D020360:Neoadjuvant Therapy; D010641:Phenotype; D012008:Recurrence; D012307:Risk Factors; D033581:Stem Cell Transplantation; D013997:Time Factors; D014178:Translocation, Genetic; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor