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Systemic Treatment Options for Advanced-Stage Mycosis Fungoides and Sézary Syndrome

  • Lymphomas (MR Smith, Section Editor)
  • Published:
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Abstract

Purpose of Review

Cutaneous T-cell lymphoma (CTCL) is a rare form of non-Hodgkin lymphoma. Globally, the most common subtypes of CTCL are mycosis fungoides and Sézary syndrome. CTCL can confer significant morbidity and even mortality in advanced disease. Here we review the current and potential future treatments for advanced-stage CTCL.

Recent findings

Heterogeneity of treatment choice has been demonstrated both in US and non-US centers. Systemic treatment choice is currently guided by prognostic features, incorporating stage, immunophenotypic and molecular findings, and patient-specific factors such as age and comorbidities. Randomized controlled studies are uncommon, and the literature is composed predominantly of retrospective, cohort, and early-phase studies. International consensus guidelines are available; however, the lack of comparative trials means that there is no clear algorithmic approach to treatment.

Summary

This review article reports on the systemic treatment options in current use for advanced CTCL, and on the possible future therapies, acknowledging that an algorithmic approach is not yet forthcoming to guide treatment prioritization.

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Correspondence to Louise Photiou.

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Louise Photiou declares that she has no conflict of interest.

Carrie van der Weyden declares that she has no conflict of interest.

Christopher McCormack has received compensation from Takeda Pharmaceuticals and MSD for participation on advisory boards.

H. Miles Prince has received research funding through grants from Celgene, Takeda, Amgen, Novartis, Innate Pharma, and Eisai; has received honoraria from Celgene, Takeda, Amgen, Novartis, Innate Pharma, and Eisai; and has participated on advisory boards for Celgene, Takeda, Amgen, Novartis, Innate Pharma, and Eisai.

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Photiou, L., van der Weyden, C., McCormack, C. et al. Systemic Treatment Options for Advanced-Stage Mycosis Fungoides and Sézary Syndrome. Curr Oncol Rep 20, 32 (2018). https://doi.org/10.1007/s11912-018-0678-x

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