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A chromosomal translocation <jats:italic>t<\/jats:italic> (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\u201011 or a low Ki\u201067 may correlate with a more indolent form of MCL. The differential diagnosis of MCL includes small lymphocytic lymphoma, marginal zone lymphoma, and follicular lymphoma.<\/jats:p><\/jats:sec><jats:sec><jats:title>Risk Stratification<\/jats:title><jats:p>The MCL 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\u201067 proliferative index if available. The median OS for the low\u2010risk group was not reached (5\u2010year OS of 60%). The median OS for the intermediate risk group was 51 months and 29 months for the high risk group.<\/jats:p><\/jats:sec><jats:sec><jats:title>Risk\u2010Adapted Therapy<\/jats:title><jats:p>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 cytotoxic<\/jats:p><jats:p>Regimen followed by autologous stem cell transplantation should be considered. Rituximab maintenance after autologous stem cell transplantation has also improved the progression\u2010free and overall survival. For older symptomatic MCL patients with intermediate or high risk MIPI, combination chemotherapy with R\u2010CHOP, R\u2010Bendamustine, or a clinical trial should be considered. In addition, rituximab maintenance therapy may prolong the progression\u2010free survival. At the time of relapse, agents directed at activated pathways in MCL cells such as bortezomib (NFkB inhibitor), lenalidamide (anti\u2010angiogenesis) and Ibruitinib (Bruton's Tyrosine Kinase [BTK] inhibitor) have demonstrated excellent clinical activity in MCL patients. Autologous or allogeneic stem cell transplantation can also be considered in young patients. Clinical trials with novel agents are always a consideration for MCL patients.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ajh.24797","type":"journal-article","created":{"date-parts":[[2017,7,12]],"date-time":"2017-07-12T12:15:11Z","timestamp":1499861711000},"page":"806-813","source":"Crossref","is-referenced-by-count":187,"title":["Mantle cell lymphoma: 2017 update on diagnosis, risk\u2010stratification, and clinical management"],"prefix":"10.1002","volume":"92","author":[{"given":"Julie M.","family":"Vose","sequence":"first","affiliation":[{"name":"Division of Hematology\/Oncology University of Nebraska Medical Center Omaha Nebraska 68198\u20107680"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,7,12]]},"reference":[{"key":"e_1_2_6_2_1","first-page":"405","article-title":"Classification of non\u2010Hodgkin's lymphomas [let\u2010ter]","volume":"2","author":"Gerard\u2010Marchant 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