ABSTRACT
Definition 482 Epidemiology 482 Ontogeny 483 Morphologic characteristics 483 Architectural patterns 483 Cytologic variants 484 Bone marrow and peripheral blood 484 Spleen 485 Gastrointestinal tract 485 Histologic progression 485 Composite mantle cell lymphoma and other lymphoid 485
neoplasms Immunophenotype 485 Cyclin D1 and p27 expression 486 Cyclin D1-negative mantle cell lymphoma 486 Cytogenetic findings 486 Molecular characteristics 487
t(11;14) translocation and cyclin D1 overexpression 487 Cyclin D1 oncogenic mechanisms 488 Deregulation of cell cycle 488 Deregulation of the DNA damage response pathway 489 Other oncogenic events 490 Mantle cell lymphoma gene expression profile 490 Mantle cell lymphoma cell lines and animal models 490 Clinical characteristics and evolution 492 Prognostic parameters 492 Morphologic characteristics 492 Proliferative activity 492 Genetic aberrations 493 Molecular alterations 493 Clinical parameters 493 Treatment of mantle cell lymphoma 493 Key points 494 References 494
Mantle cell lymphoma (MCL) is a lymphoid neoplasm characterized by a monomorphic proliferation of small-to medium-sized lymphoid cells with irregular nuclei of B cell phenotype commonly coexpressing CD5.1 The genetic hallmark of this neoplasm is the t(11;14)(q13;q32) translocation that leads to a constitutive deregulation and overexpression of cyclin D1 and plays an important pathogenetic role in the development of the tumor. This entity includes different categories of lymphomas recognized in previous classifications such as the centrocytic lymphoma, lymphocytic lymphoma of intermediate differentiation, intermediate lymphocytic lymphoma and mantle zone lymphoma.2,3 The clinical behavior is very aggressive and few patients may be considered cured or reach long survival with current therapeutic protocols. Better knowledge of this disease and emergence of a new generation of drugs are facilitating the design of new
this aggressive lymphoma to conventional treatments, thereby improving the life expectancy of the patients.
