Utility of flow cytometry immunophenotyping and DNA ploidy studies for diagnosis and characterization of blood involvement in CD4+, Sezary's syndrome

• Type: Article in International Scientific Journal
• Date: 2003

Authors

Lima, M (Author) Other; Almeida, J (Author) Other; Teixeira, MDA (Author) Other; Queiros, ML (Author) Other; Santos, AH (Author) Other; Fonseca, S (Author) Other; Balanzategui, A (Author) Other; Justica, B (Author) Other; Orfao, A (Author) Other

Journal

Title: Haematologica

Vol. 88

Pages: 874-887

ISSN: 0390-6078

Publisher: FERRATA STORTI FOUNDATION

Indexing

ISI Web of Knowledge - 26 Citations

Scopus - 22 Citations

Other information

Authenticus ID: P-000-FPK

Abstract (EN): Background and Objectives. The exact immunophenotypic criteria for the identification of Sezary 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 Sezary'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 Sezary's cells coexisted with large nearly tetraploid Sezary'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, Sezary'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-Vbeta families and investigate the specificity of these phenotypic abnormalities for diagnosing SS.

Language: English

Type (Professor's evaluation): Scientific

No. of pages: 14