title: Nodal Marginal Zone Large B-Cell Lymphoma with Burkitt Translocation and Complex Chromosomal Changes Associated with Overexpression of BCL2, MYC, and BCL6.
abstract: We report the first case of a nodal marginal zone large B-cell lymphoma and the first with MYC rearrangement. This high proliferation rate lymphoma (40% of cells) occurred in the bilateral cervical, axillary, and para-aortic lymph nodes of an 82 year old woman. It involved extensively her bone marrow, and was lethal. Malignant B-cells were CD10 negative, harbored Burkitt translocation, and multiple chromosomal changes including trisomies of chromosomes 3 and 18, and three copies of 8q with an intact q24 cytoband (in addition to MYC rearrangement), associated with overexpression of BCL6, BCL2, and MYC respectively. We suggest that in aggressive nodular marginal zone lymphomas (clinical picture or high proliferation rate of lymphoma cells), fluorescence in situ hybridization analysis for MYC rearrangement, with break-apart probe, and for MYC/IGH translocation, in addition to chromosome analysis, should be performed. MYC rearrangement associated with a more rapid progression of the neoplasia, might warrant a more aggressive treatment.
authors.affiliation: Department of Pathology and Laboratory Medicine, Memorial Hospital CAMC.
authors.forename: Tomislav M
authors.initials: TM
authors.lastname: Jelic
authors.forename: Tzong-Wen E
authors.initials: TW
authors.lastname: Huang
authors.forename: Pauline
authors.initials: P
authors.lastname: Brenholz
authors.forename: Oscar C
authors.initials: OC
authors.lastname: Estallila
authors.forename: Ahmed A
authors.initials: AA
authors.lastname: Khalid
authors.forename: Tajana
authors.initials: T
authors.lastname: Juranovic
authors.forename: Todd J
authors.initials: TJ
authors.lastname: Kuenstner
mesh_terms: D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002869:Chromosome Aberrations; D004268:DNA-Binding Proteins / Q000235:genetics*; D017809:Fatal Outcome; D005260:Female; D016259:Genes, myc; D006801:Humans; D008198:Lymph Nodes / Q000473:pathology*; D018442:Lymphoma, B-Cell, Marginal Zone / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics*; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000235:genetics*; D051560:Proto-Oncogene Proteins c-bcl-6; D014178:Translocation, Genetic
chemical_list: C084103:BCL6 protein, human; D004268:DNA-Binding Proteins; D019253:Proto-Oncogene Proteins c-bcl-2; D051560:Proto-Oncogene Proteins c-bcl-6