Marginal zone lymphoma (MZL) denominates a category of indolent B lymphocytic lymphomas engendered from the marginal zone of lymphoid follicles. Marginal zone lymphomas comprise of an estimated (8%) of non-Hodgkin’s lymphomas with a median age of disease emergence at 60 years and a slight female preponderance. Marginal zone lymphomas display identical morphological and immune phenotypic features across the subcategories. The world health organization (WHO) designates marginal zone lymphoma into three distinct subtypes as extra nodal marginal zone lymphoma (mucosa associated lymphoid tissue - MALT lymphoma), nodal marginal zone lymphoma and splenic marginal zone lymphoma. Primary nodal marginal zone lymphoma remains an exceptional entity and necessitates a demarcation from secondary nodal marginal zone lymphoma on account of metastasis from extra nodal or splenic marginal zone lymphoma. Nodal marginal zone lymphoma constitutes an estimated 2.4% and splenic marginal zone lymphoma around 0.7% of the B lymphocytic lymphomas.
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