Sézary Syndrome: Clinical and Biological Aspects

Curr Hematol Malig Rep. 2016 Oct 4.
Sézary Syndrome: Clinical and Biological Aspects.
Kohnken R1, Fabbro S2, Hastings J2, Porcu P3,4, Mishra A5,6.
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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