Document Detail


TCRgamma-chain gene rearrangement by GeneScan: incidence and significance of clonal heterogeneity in Sézary syndrome.
MedLine Citation:
PMID:  20410915     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
GeneScan (GS) analysis is a highly sensitive method for the early detection of cutaneous T-cell lymphoma (CTCL) and allows the identification of clonal heterogeneity, defined as the coexistence of two or more different T-cell clones in multiple samples from the same patient. We analyzed by GS the incidence and the significance of long-lived oligoclonal expansions in multiple skin and blood samples from 24 Sézary syndrome (SS) patients, and tried to correlate them with the clinical outcome. A skin clonal heterogeneity with additional reproducible TCRgamma-gene rearrangements (TCRgamma-GRs) was detected at diagnosis in 19/24 patients, 13 of whom had a constant prevalence of pathological TCRgamma-GRs in both skin and blood (dominant clonal pattern). During follow-up, an increase in oligoclones that were present at diagnosis or the appearance of new oligoclones was observed in 10 patients; all of them achieved a clinical response to treatment with extracorporeal photochemotherapy (ECP). The TCRgamma pattern (homogeneity or heterogeneity) in the skin at diagnosis showed a relevant prognostic value, and patients with an oligoclonal pattern had a significantly longer survival than those with a homogeneous pattern. In conclusion, multiple-sample approach GS analysis allows the identification of clonal heterogeneity and could also help in identifying SS patients with a potential higher response to therapy.
Authors:
Maria T Fierro; Renata Ponti; Stefano Titli; Lisa Bonello; Alessandra Comessatti; Mauro Novelli; Paolo Fava; Paola Francia di Celle; Pietro Quaglino; Maria Grazia Bernengo
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Publication Detail:
Type:  Journal Article     Date:  2010-04-22
Journal Detail:
Title:  The Journal of investigative dermatology     Volume:  130     ISSN:  1523-1747     ISO Abbreviation:  J. Invest. Dermatol.     Publication Date:  2010 Sep 
Date Detail:
Created Date:  2010-08-16     Completed Date:  2010-09-16     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0426720     Medline TA:  J Invest Dermatol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  2312-9     Citation Subset:  IM    
Affiliation:
Section of Dermatology, Department of Biomedical Sciences and Human Oncology, University of Turin, Turin, Italy. mariateresa.fierro@unito.it
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MeSH Terms
Descriptor/Qualifier:
Follow-Up Studies
Gene Rearrangement*
Genetic Heterogeneity
Genetic Predisposition to Disease / epidemiology
Genetic Testing / methods*
Humans
Incidence
Polymerase Chain Reaction / methods*
Prevalence
Prognosis
Receptors, Antigen, T-Cell, gamma-delta / genetics*
Sezary Syndrome* / diagnosis,  genetics,  mortality
Chemical
Reg. No./Substance:
0/Receptors, Antigen, T-Cell, gamma-delta

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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