Abstract
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.
Highlights
Nodal marginal zone lymphomaThe exceptional neoplasm constitutes an estimated 2% of the B cell lymphomas with a majority of the subjects presenting with clinical stage III or IV
Histopathology and disease outcome of the neoplasm may be insufficiently elucidated as nodal marginal zone lymphoma has been described as a separate entity by the world health organization since 2008 [3,4]
A relationship with marginal zone (MZ) B lymphocytes has been claimed for this B cell subpopulation based mainly on the architectural localization and cytology, and on the proposed immunoglobulin M (IgM)+ or immunoglobulin D (IgD)- immune phenotype and the results of mutational analysis of the rearranged immunoglobulin variable genes
Summary
The exceptional neoplasm constitutes an estimated 2% of the B cell lymphomas with a majority of the subjects presenting with clinical stage III or IV. Nodal marginal zone lymphoma may characteristically be engendered from the lymph node follicles. It may partially recapitulate morphological, immune phenotypic and genetic manifestations of an extra nodal rather than a splenic marginal zone lymphoma [1,2,3]. The contemporary classification of world health organization (WHO) defines the nodal variant as a primary nodal B cell neoplasm morphologically identical to a lymph node with attributes of a marginal zone lymphoma of the extra-nodal or splenic subtype in the absence of splenic or extranodal disease. Histopathology and disease outcome of the neoplasm may be insufficiently elucidated as nodal marginal zone lymphoma has been described as a separate entity by the world health organization since 2008 [3,4]. Nodal marginal zone lymphoma may recapitulate follicular lymphoma the disease may relapse frequently with increasingly aggressive reoccurrences
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