Immunoglobulin Gene Mutation Patterns And Heterogeneity Of Marginal Zone Lymphoma Pdf

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The considerable heterogeneity in morphology, immunophenotype, genotype, and clinical behavior of splenic marginal zone lymphoma SMZL hinders firm conclusions on the origin and differentiation stage of the neoplastic cells. These results provide evidence for the diverse B-cell subpopulations residing in the SMZ, which could represent physiologic equivalents of distinct SMZL subtypes. The marginal zone of the human spleen SMZ is a microanatomical site at the border of the white and red pulp, mainly comprising of B cells, T cells, and macrophages.

Marginal zone B-cell lymphoma

Oncotarget a primarily oncology-focused, peer-reviewed, open access, biweekly journal aims to maximize research impact through insightful peer-review; eliminate borders between specialties by linking different fields of oncology, cancer research and biomedical sciences; and foster application of basic and clinical science. Its scope is unique. The term "oncotarget" encompasses all molecules, pathways, cellular functions, cell types, and even tissues that can be viewed as targets relevant to cancer as well as other diseases. The term was introduced in the inaugural Editorial , Introducing OncoTarget. Sponsored Conferences. Impact Journals is a member of the Society for Scholarly Publishing. Immunoglobulin Ig gene rearrangements remain largely unmodified during the clonal expansion of neoplastic cells.

Immunoglobulin Heavy-And Light-chain Repertoire in Splenic Marginal Zone Lymphoma

Either your web browser doesn't support Javascript or it is currently turned off. In the latter case, please turn on Javascript support in your web browser and reload this page. Free to read. We studied the pattern of somatic hypermutation of the rearranged immunoglobulin heavy chain genes on 23 cases and have correlated these data with survival as well as immunophenotypic and genetic characteristics of the cases. Two-thirds of the cases show immunoglobulin gene mutations, half of which show evidence of antigen selection, whereas one-third of the cases show no significant mutations. No statistical significant correlation was found between immunoglobulin mutation status and clinical, immunophenotypic, or genetic characteristics. Our results demonstrate that on-going somatic hypermutation is a prominent feature of splenic marginal zone lymphoma with circulating villous lymphocytes.


Immunoglobulin Gene Mutation Patterns and Heterogeneity of Marginal Zone Beside the so-called extranodal B-cell MALT lymphoma, the splenic MZ.


Analysis of VH genes in marginal zone lymphoma reveals marked heterogeneity between splenic and nodal tumors and suggests the existence of clonal selection. Three cases out of 18 with clones analyzed from spleen and peripheral blood demonstrated intra-clonal diversity, with evidence of clonal selection in one case, indicating the possibility of antigen-driven clonal expansion. No differences in clinical outcome and overall survival were found between the unmutated and mutated cases. Moreover, a biased usage of certain sequences suggests that tumor cells in SMZL may be subjected to antigen selection. Article Information Vol.

The precise B cell of origin and molecular pathogenesis of nodal marginal zone lymphoma NMZL remain poorly defined.

The clonotypic B cell receptor immunoglobulin BcR IG plays a seminal role in B cell lymphoma development and evolution. This clinical development complements immunogenetic evidence for antigen drive in the natural history of these tumors. Moreover, distinct entities display imprints of somatic hypermutation within the clonotypic BcR IG gene following patterns that strengthen the argument for antigen selection. Of note, at least in certain B cell lymphomas, the BcR IG genes are intraclonally diversified, likely in a context of ongoing interactions with antigen s. Moreover, BcR IG gene repertoire profiling suggests that unique immune pathways lead to distinct B cell lymphomas through targeting cells at different stages in the B cell differentiation trajectory e. Regarding the implicated antigens, although their precise nature remains to be fully elucidated, immunogenetic analysis has offered important hints by revealing similarities between the BcR IG of particular lymphomas and B cell clones with known antigenic specificity: this has paved the way to functional studies that identified relevant antigenic determinants of classes of structurally similar epitopes.

Lebien , Fates of human 8-cell precursors , Blood , vol. Schwickert , ln vivo imaging of germinal centres reveals a dynamic open structure , Nature , issue. Lebien and T. DOI :