Mantle Cell Lymphoma
C Cameron Yin, MD, PhD
Key Facts
Terminology
Clinically aggressive B-cell lymphoma associated with t(11;14)(q13;q32) and Cyclin-D1 overexpression
Clinical Issues
Elderly people; male predominance
Most patients present with clinical stage III/IV disease
B symptoms, lymphadenopathy, extranodal involvement
Currently considered incurable with median survival of 2-5 years
Microscopic Pathology
Architectural effacement by lymphoma with nodular, diffuse, or mantle zone growth pattern
Monotonous population of small/medium-sized cells with variably irregular nuclear contours
Ancillary Tests
Immunohistochemistry: Cyclin-D1(+)
Flow cytometry: CD5(+), CD19(+), CD20(+), CD43(+/-), FMC-7(+), CD10(-), CD23(-)
Cytogenetics: t(11;14)(q13;q32) or CCND1-IgH (FISH)
Gene expression profiling
Unique profile
Proliferation predicts prognosis
Top Differential Diagnoses
Chronic lymphocytic leukemia/small lymphocytic lymphoma
Follicular lymphoma
Nodal marginal zone B-cell lymphoma
 Mantle cell lymphoma (MCL) with a nodular and diffuse pattern associated with hyalinized blood vessels. Hyalinized blood vessels are common in MCL. |
 Cyclin-D1 immunostain shows nuclear expression in the cells of mantle cell lymphoma. As demonstrated here, the nuclei often show cell to cell variation in the intensity of Cyclin-D1 expression. |
TERMINOLOGY
Abbreviations
Mantle cell lymphoma (MCL)
Synonyms
Centrocytic lymphoma
Lymphocytic lymphoma, intermediate grade of differentiation
Intermediate lymphocytic lymphoma
Definitions
Clinically aggressive B-cell lymphoma usually composed of monomorphic small to medium-sized cells and associated with t(11;14)/CCND-1-IgH
ETIOLOGY/PATHOGENESIS
t(11;14)(q13;q32)
Juxtaposes CCND-1 at 11q13 with IgH at 14q32 and results in Cyclin-D1 overexpression, Rb phosphorylation, and release of E2F
Facilitates cell cycle progression from G1 to S phase
CLINICAL ISSUES
Presentation
Median age: 6th-7th decades; male predominance
Most patients present with Ann Arbor clinical stage III/IV; B symptoms in 40-50%
Lymphadenopathy, generalized
Extranodal sites are common, particularly GI tract, bone marrow, spleen, peripheral blood
Treatment
Aggressive chemotherapy ± stem cell transplantation
HyperCVAD chemotherapy regimen used at many medical centers
