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Blood, 15 May 2006, Vol. 107, No. 10, pp. 4030-4038.
Prepublished online as a Blood First Edition Paper on January 17, 2006; DOI 10.1182/blood-2005-10-4239.
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NEOPLASIA
Significance of circulating T-cell clones in Sézary syndrome
Nicolas Ortonne,
Delphine Huet,
Caroline Gaudez,
Anne Marie-Cardine,
Valérie Schiavon,
Martine Bagot,
Philippe Musette, and
Armand Bensussan
From the Institut National de la Santé et de la Recherche Médicale (INSERM) Unit 659, Faculté de Médecine de Créteil, Créteil, France; Department of Pathology, Hôpital Henri Mondor, Créteil, France; INSERM Unit 532, Hôpital Saint Louis, Paris, France; Department of Dermatology, Hôpital Henri Mondor, Créteil, France; INSERM Unit 519, Faculté de Médecine de Rouen, Rouen, France; and Department of Dermatology, CHU de Rouen, Rouen, France.
Identification of malignant Sézary cells by T-cell receptor (TCR) clonality studies is routinely used for the diagnosis of Sézary syndrome, but T-cell clones expressed in a single patient have never been accurately characterized. We previously reported that CD158k expression delineates Sézary syndrome malignant cells, and, more recently, we identified vimentin at the surface membranes of Sézary cells and normal activated lymphocytes. In the present study, T-cell clones from 13 patients with Sézary syndrome were identified by immunoscopy and further characterized in the blood according to their TCR V , CD158k, and vimentin cell-surface expression. We found in most patients a unique malignant T-cell clone that coexpressed CD158k and vimentin and that, when patients were tested, was also present in the skin. However, in some patients we detected the presence of a nonmalignant circulating clone expressing high amounts of vimentin and lacking CD158k. These results indicate that clonal expansion may originate from circulating malignant and nonmalignant CD4+ T cell populations in patients with Sézary syndrome. Identification of the malignant cells in Sézary syndrome cannot be achieved by T-cell clonality studies or by TCR V monoclonal antibody (mAb) analysis alone; it also relies on CD158k phenotyping.

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