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). Procedure Flow cytometric analysis incl...

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Veröffentlicht in:Pediatric Blood & Cancer 2004-03, Vol.42 (3), p.275-277
Hauptverfasser: Boeckx, Nancy, Uyttebroeck, Anne, Langerak, Anton W., Brusselmans, Caroline, Goossens, Willy, Bossuyt, Xavier
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container_end_page 277
container_issue 3
container_start_page 275
container_title Pediatric Blood & Cancer
container_volume 42
creator Boeckx, Nancy
Uyttebroeck, Anne
Langerak, Anton W.
Brusselmans, Caroline
Goossens, Willy
Bossuyt, Xavier
description 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). Procedure Flow cytometric analysis including TCR‐Vβ 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 Vβ3 clonal nature. Molecular studies identified a clonal Vβ3‐Cβ 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. © 2003 Wiley‐Liss, Inc.
doi_str_mv 10.1002/pbc.10378
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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). Procedure Flow cytometric analysis including TCR‐Vβ 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 Vβ3 clonal nature. Molecular studies identified a clonal Vβ3‐Cβ 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. 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Blood Cancer</addtitle><description>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). Procedure Flow cytometric analysis including TCR‐Vβ 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 Vβ3 clonal nature. Molecular studies identified a clonal Vβ3‐Cβ 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. 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Cancer</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Boeckx, Nancy</au><au>Uyttebroeck, Anne</au><au>Langerak, Anton W.</au><au>Brusselmans, Caroline</au><au>Goossens, Willy</au><au>Bossuyt, Xavier</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Clonal proliferation of T-Cell large granular lymphocytes</atitle><jtitle>Pediatric Blood &amp; Cancer</jtitle><addtitle>Pediatr. 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subjects Biological and medical sciences
Child
Clone Cells - pathology
Female
Flow Cytometry
Gene Rearrangement, T-Lymphocyte
General aspects
Humans
Immunophenotyping
large granular lymphocytes
Leukemia, Lymphoid - diagnosis
Leukemia, Lymphoid - pathology
Lymphocyte Activation - physiology
Medical sciences
Molecular Diagnostic Techniques
Neutropenia - etiology
pure red blood cell aplasia
Red-Cell Aplasia, Pure - etiology
Reverse Transcriptase Polymerase Chain Reaction
T-LGL leukemia
T-Lymphocytes - pathology
TCR-Vβ analysis
Tumors
title Clonal proliferation of T-Cell large granular lymphocytes
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