mantle lymphoma | Mantle lymphoma: An organic model and CliniqueMantle cell lymphoma: a biological and therapeutic paradigm

Mantle lymphoma: An organic model and CliniqueMantle cell lymphoma: a biological and therapeutic paradigm



Summary
Recent classifications of non-Hodgkin lymphomas have individualized mantle cell lymphomas (LCM) on morphological, immunophénotypiques, and cytogenetic criteria. This entity now appears as a biological and therapeutic model in the understanding and treatment of malignant hemic. The lymphomogenèse of LCM can be explained by a series of genetic events occurring at different stages of the disease: (1) mutation and/or loss of the ATM gene (ataxia-telangiectasia mutated) in the centrocytiques cells of the follicular mantle of Lymph nodes, resulting in a loss of the function of the gene particularly involved in recombination (D) J; (2) translocation (11; 14) (q13; q32) responsible for a constitutive expression of the BCL-1/PRAD1/CCND1 protein, the latter being responsible for activating the cell cycle of centrocytiques cells characteristic of mantle lymphomas Typical (3) Secondary chromosomal anomalies, such as p53 mutation, found in the blastic forms of the LCM. Despite the evaluation of multiple therapeutic approaches, the optimal modalities for the management of LCM remain to be defined: (1) The combination of conventional and intensive chemotherapy associated with rituximab significantly increases the Response rates, as well as overall and non-recurrence survival; (2) Innovative therapeutic approaches being evaluated. From this point of view, Mantle lymphomas appear as a biological and clinical model of global hematologic reflection.

Abstract
Recent classifications of Hodgkin's lymphomas (NHL) have strictly individualized mantle cell lymphoma (MCL) on the basis of a combination of morphologic, immunophenotypic, and cytogenetic criteria. This clinicopathological entity now appears to be a biological and therapeutic model for the understanding and treatment of hematological malignancies. The lymphomogenesis of MCL could be explained by a series of genetic abnormalities which occur at different steps of the disease: (1) mutation and/or loss of the ATM gene in centrocytic cells of the follicle mantle of lymph nodes, leading to the loss of ATM function , particularly involved during the (D) J recombination process; (2) A (11; 14) (q13; q32) translocation which induces a constitutive Bcl-1/PRAD1/CCND1 expression, responsible for cell cycle activation of centrocytic cells characteristic of typical MCL; and (3) secondary additional chromosomal aberrations, such as a p53 mutation, observed in blastic transformation of MCL. Despite the evaluation of a number of treatment modalities, the optimal management of MCL has not been defined yet; (1) Conventional and intensified chemotherapy and monoclonal anti-CD20 antibody therapy appear to be effective for the improvement of response rates and event-free or overall survival; (2) Combinations of different treatment modalities must be tested to modify the natural dismal outcome of the disease; (3) Innovative approaches should be developed. From this point of view, all these considerations offer a fine opportunity for extensive medical reflection.