title: Splenic marginal-zone lymphoma: ontogeny and genetics.
abstract: Splenic marginal-zone lymphoma (SMZL) is a rare tumor that has recently emerged as a prototype for how the interplay between genetics and environment shapes the natural history of lymphomas. Indeed, the recent identification of molecular immunogenetic subgroups within SMZL may prove to be relevant not only for the sub-classification of the disease but also for improved understanding of the underlying biology. In contrast to other B-cell lymphomas, SMZL lacks a characteristic genetic lesion, although the majority of cases harbor genomic aberrations, as recently revealed by high-throughput studies that identified recurrent genetic aberrations, several in pathways related to marginal-zone differentiation and B-cell signaling. Here we provide an overview of recent research into the molecular and cellular biology of SMZL and related disorders, with special emphasis on immunogenetics and genomic aberrations, and discuss the value of molecular and cellular markers for the diagnosis and differential diagnosis of these entities.
authors.affiliation: Department of Immunology, Genetics and Pathology, Science for Life laboratory, Uppsala University , Uppsala , Sweden.
authors.forename: Panagiotis
authors.initials: P
authors.lastname: Baliakas
authors.forename: Jonathan C
authors.initials: JC
authors.lastname: Strefford
authors.forename: Vasilis
authors.initials: V
authors.lastname: Bikos
authors.forename: Marina
authors.initials: M
authors.lastname: Parry
authors.forename: Kostas
authors.initials: K
authors.lastname: Stamatopoulos
authors.forename: David
authors.initials: D
authors.lastname: Oscier
mesh_terms: D001402:B-Lymphocytes / Q000378:metabolism* / Q000473:pathology; D002869:Chromosome Aberrations; D056915:DNA Copy Number Variations; D020022:Genetic Predisposition to Disease / Q000235:genetics*; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000235:genetics* / Q000473:pathology; D009154:Mutation; D015398:Signal Transduction / Q000235:genetics; D013160:Splenic Neoplasms / Q000235:genetics* / Q000473:pathology
keywords: Lymphoma and Hodgkin disease; cytogenetics; molecular genetics