DISEASE OVERVIEW: 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.
DIAGNOSIS: 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.
RISK STRATIFICATION: 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.
RISK-ADAPTED THERAPY: 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.