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World J Immunol. Mar 27, 2013; 3(1): 1-6
Published online Mar 27, 2013. doi: 10.5411/wji.v3.i1.1
Natural killer reprogramming in cutaneous T-cell lymphomas: Facts and hypotheses
Christian Schmitt, Anne Marie-Cardine, Martine Bagot, Armand Bensussan
Christian Schmitt, Anne Marie-Cardine, Martine Bagot, Armand Bensussan, INSERM U976, F-75010 Paris, France
Christian Schmitt, Anne Marie-Cardine, Martine Bagot, Armand Bensussan, Dermatology and Oncology, Laboratory of Immunology, Univ Paris Diderot, Sorbonne Paris Cité, UMRS 976, F-75475 Paris, France
Martine Bagot, AP-HP, Hopital Saint-Louis, F-75475 Paris, France
Author contributions: Schmitt C wrote paper and designed research; Marie-Cardine A designed and performed research; Bagot M and Bensussan A supervised research and patients.
Correspondence to: Dr. Christian Schmitt, PhD, INSERM UMRS 976, 1 avenue Claude Vellefaux, F-75475 Paris, France. christian.schmitt@inserm.fr
Telephone: +33-15-3722054 Fax: +33-15-3722051
Received: April 9, 2012
Revised: January 8, 2013
Accepted: January 31, 2013
Published online: March 27, 2013

To better understand the pathogenesis of Sézary cells, distinguish them from reactive skin-infiltrating T-cells and improve disease treatment, efforts have been made to identify molecular targets deregulated by the malignant process. From immunophenotypic analysis and subtractive differential expression experiments to pan-genomic studies, many approaches have been used to identify markers of the disease. During the last decade several natural killer (NK) cell markers have been found aberrantly expressed at the surface of Sézary cells. In particular, KIR3DL2/CD158k, expressed by less than 2% of healthy individuals CD4+ T-cells, is an excellent marker to identify and follow the tumor burden in the blood of Sézary syndrome patients. It may also represent a valuable target for specific immunotherapy. Other products of the NK cluster on chromosome 19q13 have been detected on Sézary cells, raising the hypothesis of an NK reprogramming process associated with the malignant transformation that may induce survival functions.

Keywords: Sézary syndrome, Mycosis fungoides, Natural killer receptors, KIR3DL2, Cutaneous T-cell lymphomas