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Review
. 2016 Jan;91(1):151-65.
doi: 10.1002/ajh.24233. Epub 2015 Nov 26.

Cutaneous T-cell Lymphoma: 2016 Update on Diagnosis, Risk-Stratification, and Management

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Free PMC article
Review

Cutaneous T-cell Lymphoma: 2016 Update on Diagnosis, Risk-Stratification, and Management

Ryan A Wilcox. Am J Hematol. .
Free PMC article

Abstract

Disease overview: Cutaneous T-cell lymphomas are a heterogenous group of T-cell lymphoproliferative disorders involving the skin, the majority of which may be classified as Mycosis Fungoides (MF) or Sézary Syndrome (SS).

Diagnosis: The diagnosis of MF or SS requires the integration of clinical and histopathologic data.

Risk-adapted therapy: TNMB (tumor, node, metastasis, blood) staging remains the most important prognostic factor in MF/SS and forms the basis for a "risk-adapted," multidisciplinary approach to treatment. For patients with disease limited to the skin, expectant management or skin-directed therapies is preferred, as both disease-specific and overall survival for these patients is favorable. In contrast, patients with advanced-stage disease with significant nodal, visceral, or blood involvement are generally approached with biologic-response modifiers or histone deacetylase inhibitors before escalating therapy to include systemic, single-agent chemotherapy. In highly-selected patients, allogeneic stem-cell transplantation may be considered, as this may be curative in some patients.

Conflict of interest statement

Conflict of interest: Nothing to report

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