title: Splenic marginal zone lymphoma with villous lymphocytes shows on-going immunoglobulin gene mutations.
abstract: Splenic marginal zone lymphoma (also splenic lymphoma with villous lymphocytes) is a B-cell non-Hodgkin's lymphoma with a characteristic morphology and phenotype. 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. On-going mutation, a feature characteristic of follicular lymphoma, was demonstrated in all six cases randomly selected for this analysis, including one case with a low number of mutations (<2%). 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. On-going somatic hypermutation has previously been demonstrated in extra-nodal and nodal marginal zone lymphoma. Our results indicate that marginal zone lymphomas at different anatomical localizations may derive from a similar B-cell subset.
authors.affiliation: Departments of Pathology and Tumor Biology, The Norwegian Cancer Institute and Radiumhospital, University of Oslo, Oslo, Norway.
authors.forename: Anne
authors.initials: A
authors.lastname: Tierens
authors.forename: Jan
authors.initials: J
authors.lastname: Delabie
authors.forename: Agnieszka
authors.initials: A
authors.lastname: Malecka
authors.forename: Junbai
authors.initials: J
authors.lastname: Wang
authors.forename: Alicja
authors.initials: A
authors.lastname: Gruszka-Westwood
authors.forename: Daniel
authors.initials: D
authors.lastname: Catovsky
authors.forename: Estella
authors.initials: E
authors.lastname: Matutes
mesh_terms: D000368:Aged; D015703:Antigens, CD / Q000276:immunology; D018076:DNA, Complementary / Q000235:genetics; D005260:Female; D015321:Gene Rearrangement; D005803:Genes, Immunoglobulin; D018095:Germ-Line Mutation; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics*; D007135:Immunoglobulin Variable Region / Q000235:genetics; D016130:Immunophenotyping; D017404:In Situ Hybridization, Fluorescence; D008214:Lymphocytes / Q000276:immunology / Q000473:pathology; D016393:Lymphoma, B-Cell / Q000276:immunology* / Q000473:pathology; D008297:Male; D009154:Mutation; D010641:Phenotype; D012333:RNA, Messenger / Q000235:genetics / Q000302:isolation & purification; D013160:Splenic Neoplasms / Q000276:immunology* / Q000473:pathology; D013601:T-Lymphocytes / Q000276:immunology
chemical_list: D015703:Antigens, CD; D018076:DNA, Complementary; D007143:Immunoglobulin Heavy Chains; D007135:Immunoglobulin Variable Region; D012333:RNA, Messenger