title: Frequent expression of multiple myeloma 1/interferon regulatory factor 4 in Burkitt lymphoma.
abstract: Burkitt lymphoma is a highly aggressive non-Hodgkin lymphoma with endemic, sporadic, and immunodeficiency-associated clinical variants composed of monomorphic medium-sized B cells with a high proliferation rate and a translocation involving the C-MYC locus. Classically, the immunophenotype of Burkitt lymphoma has been considered to be the germinal center type. In most reports, all cases of Burkitt lymphoma are reported to be multiple myeloma 1-negative. multiple myeloma 1 expression is seen in plasma cells and in a small fraction of B cells located in the light zone of germinal centers corresponding to the final step of intra-germinal center B-cell differentiation, and in activated T cells. Therefore, multiple myeloma 1 expression may denote the final step of intra-germinal center B-cell differentiation at the centrocyte stage, as well as the subsequent steps of B-cell maturation toward plasma cells. Unlike most normal germinal center B cells, in which the expression of multiple myeloma 1 and bcl-6 are mutually exclusive, the tumor cells in approximately 50% of multiple myeloma 1-positive DLBCL show coexpression of bcl-6, suggesting that the expression of these proteins may be deregulated. Twenty-five Burkitt lymphoma cases, including 19 associated with HIV, were reported in one of the few studies in the literature; 2 of these cases showed occasional multiple myeloma 1-positive cells, less than the 20% cutoff for positivity. We studied 222 cases of well-characterized Burkitt lymphoma with the classic phenotype and C-MYC translocation and found 90 cases (40.5%) with multiple myeloma 1 nuclear expression, suggesting a late germinal center stage of differentiation.
authors.affiliation: Consultoria em Patologia, Botucatu, Sao Paulo18602-010, Brazil.
authors.forename: Gabriela
authors.initials: G
authors.lastname: Gualco
authors.forename: Eduardo M
authors.initials: EM
authors.lastname: Queiroga
authors.forename: Lawrence M
authors.initials: LM
authors.lastname: Weiss
authors.forename: Claudete E N
authors.initials: CE
authors.lastname: Klumb
authors.forename: William J
authors.initials: WJ
authors.lastname: Harrington
authors.forename: Carlos E
authors.initials: CE
authors.lastname: Bacchi
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002051:Burkitt Lymphoma / Q000235:genetics / Q000378:metabolism* / Q000473:pathology*; D002454:Cell Differentiation / Q000502:physiology; D002648:Child; D002675:Child, Preschool; D004268:DNA-Binding Proteins / Q000096:biosynthesis; D005260:Female; D018858:Germinal Center / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D007223:Infant; D050835:Interferon Regulatory Factors / Q000096:biosynthesis*; D008297:Male; D008875:Middle Aged; D051560:Proto-Oncogene Proteins c-bcl-6; D012189:Retrospective Studies; D053668:Syndecan-1 / Q000096:biosynthesis; D046888:Tissue Array Analysis; D000099218:Interferon Regulatory Factor-4
chemical_list: D004268:DNA-Binding Proteins; D050835:Interferon Regulatory Factors; D051560:Proto-Oncogene Proteins c-bcl-6; D053668:Syndecan-1; D000099218:Interferon Regulatory Factor-4; C084103:BCL6 protein, human