Clonal proliferation of T-Cell large granular lymphocytes.
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.
Boeckx
Nancy
N
Department of Laboratory Medicine, Laboratory of Haematology, University Hospital Gasthuisberg, Leuven, Belgium. nancy.boeckx@uz.kuleuven.ac.be
Uyttebroeck
Anne
A
Langerak
Anton W
AW
Brusselmans
Caroline
C
Goossens
Willy
W
Bossuyt
Xavier
X
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