Immunoglobulin Gene Mutation Patterns And Heterogeneity Of Marginal Zone Lymphoma Pdf
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The precise B cell of origin and molecular pathogenesis of nodal marginal zone lymphoma NMZL remain poorly defined.
Marginal zone B-cell lymphoma
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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. Tags Type your tag names separated by a space and hit enter. Mol Med. Belessi C.
Blood ; 17 : — Nodal marginal zone lymphoma NMZL is a rare form of indolent small B-cell lymphoma which has only been clearly identified in the last 2 decades and which to date remains incurable. Progress in therapeutic management has been slow, largely due to the very small number of patients treated and the heterogeneity of treatments administered; thus, standard-of-care treatment is currently nonspecific for this lymphoma entity. In this review, treatments routinely used to manage adult NMZL patients are presented, principally based on immunochemotherapy when treatment is needed.
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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.
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. The SMZ appears especially well equipped for rapid humoral immune responses to blood-borne antigens.
AIM : To investigate how t 11;18 q21;q21 -positive gastrointestinal MALT lymphomas relate to other marginal zone lymphomas with respect to the somatic mutation pattern of the V H genes and the expression of the marker CD All cases were immunostained for CD These data suggest that the translocation t 11;18 q21;q21 can target either B-cells at different stages of differentiation or naive B-cells that retain the capacity to differentiate upon antigen stimulation. All cases but one displayed weak to strong CD27 expression which did not correlate with the V H gene mutation status. These tumors typically arise at sites normally devoid of lymphoid tissue, which have accumulated lymphoid tissue in the context of long-standing antigenic stimulation resulting from causes such as chronic bacterial infection [ Helicobacter pylori H. Several genetic aberrations have been identified in MALT lymphomas, some of which appear to be specific for, or at least closely related to, this type of lymphoma. The oncogenic activity of the 3 translocations t 1;14 p22;q32 , t 14;18 q32;q21 and t 11;18 q21;q21 is linked to the physiological role of the BCL10 and MALT1 proteins in the antigen receptor-mediated activation of NF-kB, which is the transcription factor of a number of survival- and proliferation-related genes in B cells [ 6 , 7 ].
Immunoglobulin Gene Mutation Patterns and Heterogeneity of Marginal Zone Beside the so-called extranodal B-cell MALT lymphoma, the splenic MZ.
Marginal zone B-cell lymphomas , also known as marginal zone lymphomas MZLs , are a heterogeneous group of lymphomas that derive from the malignant transformation of marginal zone B-cells. MALT , the spleen , or lymph nodes. Mucosa-associated lymphoid tissue is a diffuse system of small concentrations of lymphoid tissue found in various submucosal membrane sites of the body such as the gastrointestinal tract , mouth , nasal cavity , pharynx , thyroid gland , breast , lung , salivary glands , eye , skin and the human spleen. However, NMZL carries a somewhat worse long term outcome than the other subtypes  and any of the MZL subtypes may progress in a low percentage of cases to a more aggressive lymphoma, particularly diffuse large B-cell lymphoma. Regardless of subtype, these EMZLs share similar pathophysiological i. However, the subtypes differ in presentation, progression, severity, treatment, and instigating factors. The following two sections describe the common pathophysiologic and histopathologic features found in all EMZL subtypes.
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.
Blood ; 10 : —