Evaluation of immunophenotypic and molecular biomarkers for Sézary syndrome using standard operating procedures

• Verfasst von: Boonk, Stéphanie E. [VerfasserIn]
• Verfasst von: Booken, Nina [VerfasserIn]
• Verfasst von: Felcht, Moritz [VerfasserIn]
• Verfasst von: Klemke, Claus-Detlev [VerfasserIn]
• Titel: Evaluation of immunophenotypic and molecular biomarkers for Sézary syndrome using standard operating procedures
• Titelzusatz: a multicenter study of 59 patients
• Verf.angabe: Stephanie E. Boonk, Willem H. Zoutman, Anne Marie-Cardine, Leslie van der Fits, Jacoba J. Out-Luiting, Tracey J. Mitchell, Isabella Tosi, Stephen L. Morris, Blaithin Moriarty, Nina Booken, Moritz Felcht, Pietro Quaglino, Renata Ponti, Emanuela Barberio, Caroline Ram-Wolff, Kirsi Jäntti, Annamari Ranki, Maria Grazia Bernengo, Claus-Detlev Klemke, Armand Bensussan, Laurence Michel, Sean Whittaker, Martine Bagot, Cornelis P. Tensen, Rein Willemze and Maarten H. Vermeer
• E-Jahr: 2016
• Jahr: 29 April 2016
• Umfang: 9 S.
• Fussnoten: Gesehen am 03.07.2019
• Titel Quelle: Enthalten in: The journal of investigative dermatology
• Ort Quelle: Amsterdam : Elsevier, 1938
• Jahr Quelle: 2016
• Band/Heft Quelle: 136(2016), 7, Seite 1364-1372
• ISSN Quelle: 1523-1747
• DOI: doi:10.1016/j.jid.2016.01.038
• Datenträger: Online-Ressource
• Sprache: eng
• K10plus-PPN: 1668487551

Abstract

Differentiation between Sézary syndrome and erythrodermic inflammatory dermatoses can be challenging, and a number of studies have attempted to identify characteristic immunophenotypic changes and molecular biomarkers in Sézary cells that could be useful as additional diagnostic criteria. In this European multicenter study, the sensitivity and specificity of these immunophenotypic and recently proposed but unconfirmed molecular biomarkers in Sézary syndrome were investigated. Peripheral blood CD4+ T cells from 59 patients with Sézary syndrome and 19 patients with erythrodermic inflammatory dermatoses were analyzed for cell surface proteins by flow cytometry and for copy number alterations and differential gene expression using custom-made quantitative PCR plates. Experiments were performed in duplicate in two independent centers using standard operating procedures with almost identical results. Sézary cells showed MYC gain (40%) and MNT loss (66%); up-regulation of DNM3 (75%), TWIST1 (69%), EPHA4 (66%), and PLS3 (66%); and down-regulation of STAT4 (91%). Loss of CD26 (≥80% CD4+ T cells) and/or CD7 (≥40% CD4+ T cells) and combination of altered expression of STAT4, TWIST1, and DNM3 or PLS3 could distinguish, respectively, 83% and 98% of patients with Sézary syndrome from patients with erythrodermic inflammatory dermatoses with 100% specificity. These additional diagnostic panels will be useful adjuncts in the differential diagnosis of Sézary syndrome versus erythrodermic inflammatory dermatoses.