title: BCL6 gene rearrangements also occur in marginal zone B-cell lymphoma.
abstract: Marginal zone B-cell lymphoma (MZBCL) represents a distinct subtype of B-cell non-Hodgkin's lymphoma (NHL) which has been recently recognized and defined as a disease entity. Cytogenetically, these lymphomas reveal a high prevalence of trisomy 3, and recent data obtained by comparative genomic hybridization indicate that the chromosomal regions 3q21-23 and 3q25-29 might be of particular pathogenetic significance. We identified structural chromosomal abnormalities involving the region 3q27 and rearrangements of the BCL6 proto-oncogene in three out of 34 (9%) well-defined cases of extranodal, nodal and splenic MZBCL using cytogenetic analysis. Southern blot, and fluorescence in situ hybridization (FISH). All three cases were characterized by a t(3;14)(q27;q32). Two of them showed additional chromosomal abnormalities including trisomy 3, which was found in one case. The patients displayed extranodal disease and did not demonstrate any striking clinical and histological differences when compared with MZBCL lacking BCL6 rearrangement. The present study for the first time demonstrates the occurrence of t(3;14)/BCL6 gene rearrangement in MZBCL, thus suggesting a role of the BCL6 proto-oncogene in the pathogenesis of MZBCL.
authors.affiliation: Centre for Human Genetics, University of Leuven, Belgium.
authors.forename: J
authors.initials: J
authors.lastname: Dierlamm
authors.forename: S
authors.initials: S
authors.lastname: Pittaluga
authors.forename: M
authors.initials: M
authors.lastname: Stul
authors.forename: I
authors.initials: I
authors.lastname: Wlodarska
authors.forename: L
authors.initials: L
authors.lastname: Michaux
authors.forename: J
authors.initials: J
authors.lastname: Thomas
authors.forename: G
authors.initials: G
authors.lastname: Verhoef
authors.forename: A
authors.initials: A
authors.lastname: Verhest
authors.forename: C
authors.initials: C
authors.lastname: Depardieu
authors.forename: J J
authors.initials: JJ
authors.lastname: Cassiman
authors.forename: A
authors.initials: A
authors.lastname: Hagemeijer
authors.forename: C
authors.initials: C
authors.lastname: De Wolf-Peeters
authors.forename: H
authors.initials: H
authors.lastname: Van den Berghe
mesh_terms: D000368:Aged; D015139:Blotting, Southern; D004268:DNA-Binding Proteins / Q000235:genetics*; D005260:Female; D015321:Gene Rearrangement; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D007621:Karyotyping; D016393:Lymphoma, B-Cell / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D000090063:Proto-Oncogene Mas; D011518:Proto-Oncogene Proteins / Q000235:genetics*; D051560:Proto-Oncogene Proteins c-bcl-6; D014157:Transcription Factors / Q000235:genetics*
chemical_list: D004268:DNA-Binding Proteins; C000715470:MAS1 protein, human; D000090063:Proto-Oncogene Mas; D011518:Proto-Oncogene Proteins; D051560:Proto-Oncogene Proteins c-bcl-6; D014157:Transcription Factors