Mantle Cell Lymphoma



Mantle Cell Lymphoma


C Cameron Yin, MD, PhD










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

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Mantle Cell Lymphoma
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