title: Aberrations of MYC are a common event in B-cell prolymphocytic leukemia.
abstract: OBJECTIVE
  B-cell prolymphocytic leukemia (B-PLL) remains a controversial entity, and its molecular pathogenesis is largely unknown. Patients are older, typically having marked lymphocytosis and splenomegaly in the absence of lymphadenopathy. It is defined as a mature B-cell leukemia with more than 55% circulating prolymphocytes. Leukemic mantle cell lymphoma and chronic lymphocytic leukemia in prolymphocytic transformation must be excluded.
  
  
  METHODS
  Case archives were retrospectively reviewed for B-PLL in patients without a previous diagnosis of chronic lymphocytic leukemia or other B-cell neoplasm.
  
  
  RESULTS
  We identified six cases of B-PLL with available cytogenetic data, five of which showed evidence of aberrations in MYC. Three cases showed additional signals for the MYC gene by fluorescence in situ hybridization (FISH), and two cases demonstrated t(8;14)MYC/IGH by karyotyping or FISH. High levels of MYC protein expression were detected in all cases tested with MYC aberrations.
  
  
  CONCLUSIONS
  These results suggest that deregulation of MYC plays an important role in the pathogenesis of B-PLL and expands the spectrum of B-cell neoplasms associated with aberrations of MYC.
authors.affiliation: From the Department of Pathology, Oregon Health & Science University, Portland;
authors.forename: Ellen
authors.initials: E
authors.lastname: Flatley
authors.affiliation: Department of Hematology-Oncology, Knight Cancer Institute, Oregon Health & Science University, Portland;
authors.forename: Andy I
authors.initials: AI
authors.lastname: Chen
authors.affiliation: Hematopathology Section, Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, Bethesda, MD; and.
authors.forename: Xiangrong
authors.initials: X
authors.lastname: Zhao
authors.affiliation: Hematopathology Section, Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, Bethesda, MD; and.
authors.forename: Elaine S
authors.initials: ES
authors.lastname: Jaffe
authors.affiliation: From the Department of Pathology, Oregon Health & Science University, Portland;
authors.forename: Jennifer B
authors.initials: JB
authors.lastname: Dunlap
authors.affiliation: Hematopathology Section, Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, Bethesda, MD; and.
authors.forename: Stefania
authors.initials: S
authors.lastname: Pittaluga
authors.affiliation: Hematopathology Section, Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, Bethesda, MD; and.
authors.forename: Shahed
authors.initials: S
authors.lastname: Abdullah
authors.affiliation: Knight Diagnostic Laboratory, Knight Cancer Institute, Oregon Health & Science University, Portland.
authors.forename: Susan B
authors.initials: SB
authors.lastname: Olson
authors.affiliation: Department of Hematology-Oncology, Knight Cancer Institute, Oregon Health & Science University, Portland;
authors.forename: Stephen E
authors.initials: SE
authors.lastname: Spurgeon
authors.affiliation: From the Department of Pathology, Oregon Health & Science University, Portland; fang@ohsu.edu.
authors.forename: Guang
authors.initials: G
authors.lastname: Fan
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D007621:Karyotyping; D054403:Leukemia, Prolymphocytic, B-Cell / Q000235:genetics* / Q000473:pathology; D007964:Leukocytosis / Q000235:genetics* / Q000473:pathology; D008214:Lymphocytes / Q000473:pathology; D008297:Male; D008875:Middle Aged; D016271:Proto-Oncogene Proteins c-myc / Q000235:genetics*; D012189:Retrospective Studies
chemical_list: C489427:MYC protein, human; D016271:Proto-Oncogene Proteins c-myc
keywords: Cytogenetics; FISH; Leukemia; MYC; Prolymphocytic leukemia