Utilize este identificador para referenciar este registo: http://hdl.handle.net/10400.16/566
Título: Utility of flow cytometry immunophenotyping and DNA ploidy studies for diagnosis and characterization of blood involvement in CD4+ Sezary's syndrome
Autor: Lima, M.
Almeida, J.
dos Anjos Teixeira, M.
Queiros, M.L.
Santos, A.H.
Fonseca, S.
Balanzategui, A.
Justiça, B.
Orfão, A.
Data: Ago-2003
Editora: Pensiero Scientifico / Ferrata Storti Foundation
Resumo: Haematologica. 2003 Aug;88(8):874-87. Utility of flow cytometry immunophenotyping and DNA ploidy studies for diagnosis and characterization of blood involvement in CD4+ Sézary's syndrome. Lima M, Almeida J, dos Anjos Teixeira M, Queiros ML, Santos AH, Fonseca S, Balanzategui A, Justica B, Orfao A. Serviço de Hematologia, Unidade de Citometria, Hospital Geral de Santo António, Rua D Manuel II, s/n, 4099-001 Porto, Portugal. mmc.lima@clix.pt Abstract BACKGROUND AND OBJECTIVES: The exact immunophenotypic criteria for the identification of Sézary cells in the blood are still poorly defined. DESIGN AND METHODS: We analyzed the immunophenotype and DNA cell content of blood T cells in a series of 18 consecutive cases of Sézary's syndrome (SS), 21 normal individuals and 10 patients with reactive erythroderma, and correlated them with molecular and morphological findings. RESULTS: Phenotypically abnormal CD3+/TCRalphabeta+/CD4+ T cells were found in all SS patients but in none of the reactive erythroderma cases; small diploid, or less frequently hypodiploid Sézary's cells coexisted with large nearly tetraploid Sézary's cells in some cases. The most frequent phenotypic aberrations consisted in decreased expression of CD3/TCRalphabeta (94%), CD4 (94%), CD7 (100%) and/or CD2 (83%). In addition, Sézary's cells were constantly CD28+ and CD5+ and they did not express natural-killer associated (NKa) antigens. Phenotypic heterogeneity was a common finding and phenotypic changes over time were frequently observed. In contrast to what was found in patients with reactive erythroderma, flow cytometry analysis of the T-cell receptor (TCR) repertoire revealed a major TCR-Vbeta expansion in all SS cases. INTERPRETATION AND CONCLUSIONS: The presence of CD28+/CD5+/NKa-/CD4+ T cells expressing abnormally low levels of CD3, TCRalphabeta, CD4, CD7 and/or CD2 would support the diagnosis of SS in patients with erythroderma. Further analyses on larger series of patients are necessary in order to cover less frequent phenotypic patterns, establish the preferential usage of specific TCR-Vb families and investigate the specificity of these phenotypic abnormalities for diagnosing SS. PMID: 12935975 [PubMed - indexed for MEDLINE]Free Article
Peer review: yes
URI: http://hdl.handle.net/10400.16/566
ISSN: 0390-6078
Versão do Editor: http://www.haematologica.org/cgi/reprint/88/8/874
Aparece nas colecções:SHC - Artigos publicados em revistas indexadas na Medline

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