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Sézary Syndrome: Clinical and Biological Aspects

  • T-Cell and Other Lymphoproliferative Malignancies (P Porcu, Section Editor)
  • Published:
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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.

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Authors and Affiliations

  1. Department of Veterinary Biosciences, College of Veterinary Medicine, The Ohio State University, Columbus, OH, USA

    Rebecca Kohnken

  2. Division of Dermatology, Department of Internal Medicine, The Ohio State University, Columbus, OH, USA

    Stephanie Fabbro, Justin Hastings & Anjali Mishra

  3. Comprehensive Cancer Center, James Cancer Hospital and Solove Research Institute, The Ohio State University, Columbus, OH, USA

    Pierluigi Porcu & Anjali Mishra

  4. Division of Hematology, Department of Internal Medicine, The Ohio State University, Columbus, OH, USA

    Pierluigi Porcu

Authors
  1. Rebecca Kohnken
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  2. Stephanie Fabbro
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  3. Justin Hastings
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  4. Pierluigi Porcu
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  5. Anjali Mishra
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Correspondence to Pierluigi Porcu or Anjali Mishra.

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Rebecca Kohnken, Stephanie Fabbro, Justin Hastings, and Anjali Mishra each declare no potential conflict of interest.

Pierluigi Porcu is a section editor for Current Hematologic Malignancy Reports.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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This article is part of the Topical Collection on T-Cell and Other Lymphoproliferative Malignancies

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Kohnken, R., Fabbro, S., Hastings, J. et al. Sézary Syndrome: Clinical and Biological Aspects. Curr Hematol Malig Rep 11, 468–479 (2016). https://doi.org/10.1007/s11899-016-0351-0

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