title: Clonal proliferation of T-Cell large granular lymphocytes.
abstract: BACKGROUND
  T-cell large granular lymphocyte (T-LGL) leukemia is a disorder only rarely reported in children. We diagnosed a new case of a clonal T-LGL proliferation in a 6-year-old girl presenting with severe neutropenia and pure red blood cell aplasia (PRCA).
  
  
  METHODS
  Flow cytometric analysis including TCR-Vbeta repertoire analysis and molecular studies using reverse transcriptase polymerase chain reaction (RT-PCR), PCR heteroduplex analysis and GeneScan analysis were performed to investigate the clonal nature of the T-LGL.
  
  
  RESULTS
  Flow cytometric analysis revealed a Vbeta3 clonal nature. Molecular studies identified a clonal Vbeta3-Cbeta RT-PCR product. Both PCR heteroduplex analysis and GeneScan analysis found clonal TCRB and TCRG gene rearrangements.
  
  
  CONCLUSIONS
  An underlying T-LGL leukemia should be investigated in the diagnostic evaluation of acquired PRCA and neutropenia in young children. Both flow cytometric and molecular analyses can be used to establish the clonal nature of T-LGL.
authors.affiliation: Department of Laboratory Medicine, Laboratory of Haematology, University Hospital Gasthuisberg, Leuven, Belgium. nancy.boeckx@uz.kuleuven.ac.be
authors.forename: Nancy
authors.initials: N
authors.lastname: Boeckx
authors.forename: Anne
authors.initials: A
authors.lastname: Uyttebroeck
authors.forename: Anton W
authors.initials: AW
authors.lastname: Langerak
authors.forename: Caroline
authors.initials: C
authors.lastname: Brusselmans
authors.forename: Willy
authors.initials: W
authors.lastname: Goossens
authors.forename: Xavier
authors.initials: X
authors.lastname: Bossuyt
mesh_terms: D002648:Child; D002999:Clone Cells / Q000473:pathology; D005260:Female; D005434:Flow Cytometry; D015329:Gene Rearrangement, T-Lymphocyte; D006801:Humans; D016130:Immunophenotyping; D007945:Leukemia, Lymphoid / Q000175:diagnosis / Q000473:pathology*; D008213:Lymphocyte Activation / Q000502:physiology*; D025202:Molecular Diagnostic Techniques; D009503:Neutropenia / Q000209:etiology; D012010:Red-Cell Aplasia, Pure / Q000209:etiology; D020133:Reverse Transcriptase Polymerase Chain Reaction; D013601:T-Lymphocytes / Q000473:pathology