title: Molecular pathogenesis of splenic and nodal marginal zone lymphoma.
abstract: Genomic studies have improved our understanding of the biological basis of splenic (SMZL) and nodal (NMZL) marginal zone lymphoma by providing a comprehensive and unbiased view of the genes/pathways that are deregulated in these diseases. Consistent with the physiological involvement of NOTCH, NF-κB, B-cell receptor and toll-like receptor signaling in mature B-cells differentiation into the marginal zone B-cells, many oncogenic mutations of genes involved in these pathways have been identified in SMZL and NMZL. Beside genetic lesions, also epigenetic and post-transcriptional modifications contribute to the deregulation of marginal zone B-cell differentiation pathways in SMZL and NMZL. This review describes the progress in understanding the molecular mechanism underlying SMZL and NMZL, including molecular and post-transcriptional modifications, and discusses how information gained from these efforts has provided new insights on potential targets of diagnostic, prognostic and therapeutic relevance in SMZL and NMZL.
authors.affiliation: Hematology, Institute of Oncology Research and Oncology Institute of Southern Switzerland, Bellinzona, Switzerland.
authors.forename: Valeria
authors.initials: V
authors.lastname: Spina
authors.affiliation: Hematology, Institute of Oncology Research and Oncology Institute of Southern Switzerland, Bellinzona, Switzerland. Electronic address: davide.rossi@eoc.ch.
authors.forename: Davide
authors.initials: D
authors.lastname: Rossi
mesh_terms: D001402:B-Lymphocytes / Q000378:metabolism; D002454:Cell Differentiation; D044127:Epigenesis, Genetic; D015972:Gene Expression Regulation, Neoplastic; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000175:diagnosis / Q000235:genetics / Q000378:metabolism / Q000628:therapy; D009363:Neoplasm Proteins / Q000235:genetics / Q000378:metabolism; D011499:Protein Processing, Post-Translational; D013160:Splenic Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000378:metabolism / Q000628:therapy
chemical_list: D009363:Neoplasm Proteins
keywords: Mutation; NF-κB; NOTCH; Nodal marginal zone lymphoma; Splenic marginal zone lymphoma