Abstract
AbstractMarginal zone lymphoma (MZL) represent a group of B-cell lymphomas arising from marginal zone of secondary lymphoid follicules. The 2008 WHO classification of malignant hematological diseases distinguished three well –recognized entities,depending on the site of primary involvement : the extra-nodal MZL (EMZL), the splenic MZL (SMZL) and the nodal MZL (NMZL) Splenic (SMZL) and nodal (NMZL). In 2016, two provisionnal entities were added including the monoclonal B lymphocytosis with MZL features and the splenic red pulp lymphoma.MZL are rare indolent lymphomas, with distinct treatment strategies. A lot of these strategies have been developped in academic trials within the international extranodal lymphoma study group and the international splenic group. These international consortiums allowed to build projects involved in the lymphomagenesis of the heterogeneous lymphoma, and to make great progress in the diagnosis, the evaluation and the therapeutic recommendations. Novel drugs including cell therapy, bispecifique monoclonal antibodies, targeted drugs are currently evaluated. This will offer in a near future new perspective for the patients with MZL.
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