Am J Hematol. 2012 Jun;
Mantle cell lymphoma: 2012 update on diagnosis,
risk-stratification, and clinical management.
Vose JM. Source: Division of Hematology/Oncology, University of
Nebraska Medical Center, Omaha, Nebraska.
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. (median OS - meaning about half
live less long, half live longer)
Diagnosis is based on lymph node, bone marrow, or tissue morphology
of centrocytic lymphocytes, small cell type, or blastoid variant
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.
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
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 and 29 months for the high-risk group.
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), BTK inhibitors or CAL-101
(B-cell receptor inhibitors) or lenalidamide (antiangiogenesis) have
clinical activity in MCL patients. Autologous or allogeneic stem
cell transplantation can also be considered in young patients. Am.
J. Hematol. 87:604-609, 2012. © 2012 Wiley Periodicals, Inc.
Copyright © 2012 Wiley Periodicals, Inc.