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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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Submitted October 25, 2005
Accepted December 30, 2005

Significance of circulating T cells clones in Sezary syndrome

Nicolas Ortonne, Delphine Huet, Caroline Gaudez, Anne Marie-Cardine, Valerie Schiavon, Martine Bagot, Philippe Musette, and Armand Bensussan*

Faculte de Medecine de Creteil, Institut National de la Sante et de la Recherche Medicale (INSERM) Unit 659, Creteil, France; Department of Pathology, Hopital Henri Mondor, Creteil, France
Faculte de Medecine de Creteil, Institut National de la Sante et de la Recherche Medicale (INSERM) Unit 659, Creteil, France
INSERM Unit 532, Hopital Saint Louis, Paris, France
Faculte de Medecine de Creteil, Institut National de la Sante et de la Recherche Medicale (INSERM) Unit 659, Creteil, France; Department of Dermatology, Hopital Henri Mondor, Creteil, France
INSERM Unit 519, Faculte de Medecine de Rouen, Rouen, France; Department of Dermatology, CHU de Rouen, Rouen, France

* Corresponding author; email: bensussan{at}im3.inserm.fr.

Identification of malignant Sezary cells by TCR clonality studies is routinely used for Sezary syndrome diagnosis, but accurate characterization of the T-cell clones expressed in a single patient has never been done. We previously reported that CD158k expression delineates Sezary syndrome malignant cells, and more recently, we identified vimentin at the surface membrane of Sezary cells and normal activated lymphocytes. In the present study, T-cell clones from 13 Sezary syndrome patients were identified by immunoscopy and further characterized in the blood according to their TCRV{beta}, CD158k, and vimentin cell surface expression. We found in the majority of patients, a unique malignant T-cell clone that co-expressed CD158k and vimentin that was also present in the skin, when tested. However, in some patients we detected the presence of a nonmalignant circulating clone expressing high amount of vimentin and lacking CD158k. Thus, these results indicate that clonal expansion may originate from circulating malignant and non malignant CD4+ T cell population in Sezary syndrome. Identification of the malignant cells in Sezary syndrome cannot be achieved by T-cell clonality studies or by TCRV{beta} moAb analysis alone but also relies on CD158k phenotyping.


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