title: Mantle cell lymphoma: 2013 Update on diagnosis, risk-stratification, and clinical management.
abstract: BACKGROUND
  Mantle cell lymphoma (MCL) is a non-Hodgkin lymphoma characterized by involvement of the lymph nodes, spleen, blood, and bone marrow with a short remission duration to standard therapies and a median overall survival of 4-5 years.
  
  
  METHODS
  Diagnosis is based on lymph node, bone marrow, or tissue morphology of centrocytic lymphocytes, small cell type, or blastoid variant cells. A chromosomal translocation t(11:14) is the molecular hallmark of MCL, resulting in the overexpression of cyclin D1. Cyclin D1 is detected by immunohistochemistry in 98% of cases. The absence of SOX-11 or a low Ki-67 may correlate with a more indolent form of MCL. The differential diagnosis of MCL includes small lymphocytic lymphoma, marginal zone lymphoma, and follicular lymphoma.
  
  
  METHODS
  The Mantle Cell Lymphoma International Prognostic Index (MIPI) is the prognostic model most often used and incorporates ECOG performance status, age, leukocyte count, and lactic dehydrogenase. A modification of the MIPI also adds the Ki-67 proliferative index if available. The median overall survival (OS) for the low risk group was not reached (5-year OS of 60%). The median OS for the intermediate risk group was 51 months and 29 months for the high risk group.
  
  
  METHODS
  For selected indolent, low MIPI MCL patients, initial observation may be appropriate therapy. For younger patients with intermediate or high risk MIPI MCL, aggressive therapy with a cytarabine containing regimen ± autologous stem cell transplantation should be considered. For older MCL patients with intermediate or high risk MIPI, combination chemotherapy with R-CHOP, R-Bendamustine, or a clinical trial should be considered. At the time of relapse, agents directed at activated pathways in MCL cells such as bortezomib (NFkB inhibitor) or lenalidamide (anti-angiogenesis) are approved agents. Clinical trials with Ibruitinib (Bruton's Tyrosine Kinase inhibitor) or Idelalisib (PI3K inhibitor) have demonstrated excellent clinical activity in MCL patients. Autologous or allogeneic stem cell transplantation can also be considered in young patients.
authors.affiliation: Division of Hematology/Oncology, University of Nebraska Medical Center, Omaha, Nebraska.
authors.forename: Julie M
authors.initials: JM
authors.lastname: Vose
mesh_terms: D000367:Age Factors; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor; D001856:Bone Marrow Examination; D002880:Chromosomes, Human, Pair 11 / Q000648:ultrastructure; D002883:Chromosomes, Human, Pair 14 / Q000648:ultrastructure; D002986:Clinical Trials as Topic; D003131:Combined Modality Therapy; D019938:Cyclin D1 / Q000097:blood / Q000235:genetics; D019468:Disease Management; D019940:Genes, bcl-1; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D053208:Kaplan-Meier Estimate; D019394:Ki-67 Antigen / Q000032:analysis; D008221:Lymphoid Tissue / Q000473:pathology; D020522:Lymphoma, Mantle-Cell / Q000145:classification / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics / Q000473:pathology / Q000601:surgery; D008875:Middle Aged; D011379:Prognosis; D018570:Risk Assessment; D014178:Translocation, Genetic
chemical_list: D014408:Biomarkers, Tumor; C530104:CCND1 protein, human; D019394:Ki-67 Antigen; D019938:Cyclin D1