[New developments in Sézary syndrome].
Sézary syndrome (SS) represents 3% of cutaneous T-cell lymphomas (CTCL). It is an aggressive epidermotropic CTCL with a 5-year survival rate of 24%. According to EORTC (European organization for research and treatment of cancer), SS is defined by erythroderma, diffuse lymphadenopathy, atypical T lymphocytes (>1000/mm(3)), and the presence of a major blood, cutaneous and nodal T cell clone. A specific marker for atypical tumoral T lymphocytes known as Sézary cells was identified in 2001, namely KIR3DL2 (CD158k) receptor, which allows more specific diagnosis of SS; levels of this marker are highly correlated with the clinical course of the disease. In therapeutic terms, clinical trials are being conducted on new molecules that point towards an improved prognosis for this disease. We propose a review of Sézary syndrome, which is currently the subject of scientific papers concerning both physiopathology and therapeutics, with new prospects of targeted therapy.
Service de dermatologie, hôpital Sud, CHU d'Amiens, France. acaudron02@gmail.com
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Caudron
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Marie-Cardine
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Bensussan
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Bagot
D000818:Animals; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use; D017209:Apoptosis / Q000187:drug effects; D000077610:Bexarotene; D014408:Biomarkers, Tumor / Q000032:analysis; D003131:Combined Modality Therapy; D004167:Diphtheria Toxin / Q000627:therapeutic use; D004195:Disease Models, Animal; D004583:Electrons / Q000627:therapeutic use; D018380:Hematopoietic Stem Cell Transplantation; D056572:Histone Deacetylase Inhibitors / Q000627:therapeutic use; D006801:Humans; D016898:Interferon-alpha / Q000627:therapeutic use; D007376:Interleukin-2 / Q000627:therapeutic use; D051379:Mice; D016513:Mice, SCID; D058990:Molecular Targeted Therapy; D011701:PUVA Therapy; D017893:Photopheresis; D054347:Receptors, KIR3DL2 / Q000032:analysis / Q000235:genetics; D011993:Recombinant Fusion Proteins / Q000627:therapeutic use; D012751:Sezary Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology / Q000628:therapy; D012878:Skin Neoplasms / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology / Q000628:therapy; D013764:Tetrahydronaphthalenes / Q000627:therapeutic use; D035703:Therapies, Investigational
D000911:Antibodies, Monoclonal; D000970:Antineoplastic Agents; D014408:Biomarkers, Tumor; D004167:Diphtheria Toxin; D056572:Histone Deacetylase Inhibitors; D016898:Interferon-alpha; D007376:Interleukin-2; C515084:KIR3DL2 protein, human; D054347:Receptors, KIR3DL2; D011993:Recombinant Fusion Proteins; D013764:Tetrahydronaphthalenes; C078456:denileukin diftitox; D000077610:Bexarotene