title: Sézary Syndrome: Clinical and Biological Aspects.
abstract: Sézary syndrome (SS) is a rare and aggressive type of cutaneous T cell lymphoma (CTCL) characterized by an intensely pruritic, exfoliative rash, known as erythroderma, with cutaneous and systemic dissemination of clonal CD4+ T cells into the blood and lymph nodes. This review aims to present recent advancements in the biological and clinical aspects of SS. We begin by providing an overview of the diagnostic criteria for SS and reviewing some of its epidemiological and clinical aspects. We then discuss updates in the etiology of this elusive disease and the genetic and molecular landscapes that define it. Finally, we provide a short overview of the current therapeutic strategies for SS as well as recent advances in the prognosis of this disease. A brief set of recommendations is provided regarding future directions in research and therapy.
authors.affiliation: Department of Veterinary Biosciences, College of Veterinary Medicine, The Ohio State University, Columbus, OH, USA.
authors.forename: Rebecca
authors.initials: R
authors.lastname: Kohnken
authors.affiliation: Division of Dermatology, Department of Internal Medicine, The Ohio State University, Columbus, OH, USA.
authors.forename: Stephanie
authors.initials: S
authors.lastname: Fabbro
authors.affiliation: Division of Dermatology, Department of Internal Medicine, The Ohio State University, Columbus, OH, USA.
authors.forename: Justin
authors.initials: J
authors.lastname: Hastings
authors.affiliation: Comprehensive Cancer Center, James Cancer Hospital and Solove Research Institute, The Ohio State University, Columbus, OH, USA. pierluigi.Porcu@osumc.edu.|Division of Hematology, Department of Internal Medicine, The Ohio State University, Columbus, OH, USA. pierluigi.Porcu@osumc.edu.
authors.forename: Pierluigi
authors.initials: P
authors.lastname: Porcu
authors.affiliation: Division of Dermatology, Department of Internal Medicine, The Ohio State University, Columbus, OH, USA. anjali.mishra@osumc.edu.|Comprehensive Cancer Center, James Cancer Hospital and Solove Research Institute, The Ohio State University, Columbus, OH, USA. anjali.mishra@osumc.edu.
authors.forename: Anjali
authors.initials: A
authors.lastname: Mishra
mesh_terms: D015496:CD4-Positive T-Lymphocytes / Q000166:cytology / Q000378:metabolism; D004791:Enzyme Inhibitors / Q000627:therapeutic use; D057890:Epigenomics; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D016410:Lymphoma, T-Cell, Cutaneous / Q000188:drug therapy / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D009367:Neoplasm Staging; D012751:Sezary Syndrome / Q000188:drug therapy / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D015398:Signal Transduction
chemical_list: D004791:Enzyme Inhibitors; D007155:Immunologic Factors
keywords: Cutaneous T cell lymphoma; Lymphoproliferative malignancies; Sézary syndrome