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Current Hematologic Malignancy Reports
Erschienen in: Current Hematologic Malignancy Reports 6/2016

04.10.2016 | T-Cell and Other Lymphoproliferative Malignancies (P Porcu, Section Editor)

Sézary Syndrome: Clinical and Biological Aspects

verfasst von: Rebecca Kohnken, Stephanie Fabbro, Justin Hastings, Pierluigi Porcu, Anjali Mishra

Erschienen in: Current Hematologic Malignancy Reports | Ausgabe 6/2016

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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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Metadaten
Titel
Sézary Syndrome: Clinical and Biological Aspects
verfasst von
Rebecca Kohnken
Stephanie Fabbro
Justin Hastings
Pierluigi Porcu
Anjali Mishra
Publikationsdatum
04.10.2016
Verlag
Springer US
Erschienen in
Current Hematologic Malignancy Reports / Ausgabe 6/2016
Print ISSN: 1558-8211
Elektronische ISSN: 1558-822X
DOI
https://doi.org/10.1007/s11899-016-0351-0

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