Abstract

Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common subtypes of cutaneous T-cell lymphoma. The majority of MF cases present with only patches and plaques and the lesions are usually limited to the skin. On the other hand, in some cases, patients show skin tumors or erythroderma followed by lymph node involvement and rarely visceral organ involvement. SS is a rare, aggressive cutaneous T-cell lymphoma marked by exfoliative erythroderma, lymphadenopathy, and leukemic blood involvement. Because patients with relapsed or refractory MF/SS display a poor prognosis and the current treatment options are characterized by high rates of relapse, there is unmet need for the efficient treatment. This review provides a discussion of the recent and future promising therapeutic approaches in the management of advanced MF/SS. These include mogamulizumab, brentuximab vedotin, alemtuzumab, immune checkpoint inhibitors, IPH4102 (anti-KIR3DL2 antibody), histone deacetylase inhibitors (vorinostat, romidepsin, panobinostat, belinostat, and resminostat), pralatrexate, forodesine, denileukin diftitox, duvelisib, lenalidomide, and everolimus.

Highlights

  • Cutaneous T-cell lymphoma (CTCL) comprises a clinically/pathologically heterogeneous group of uncommon non-Hodgkin’s lymphomas that manifest primarily in the skin

  • Purpose of the present paper is to review the clinical results obtained in clinical trials of novel currently used and future promising therapies for advanced Mycosis fungoides (MF)/Sézary syndrome (SS) patients (Table 1)

  • It is known that the baseline expression level of CD25, which is not included in the intermediate affinity IL-2 receptor (IL-2R), on CTCL cell in lesional skin correlated with their clinical response to denileukin diftitox [71], suggesting that the high affinity IL-2R is the most important receptor to elicit an effect

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Summary

Tomonori Oka and Tomomitsu Miyagaki*

Department of Dermatology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan Reviewed by: Kazuyasu Fujii, Kagoshima University, Japan Takatoshi Shimauchi, Hamamatsu University School of Medicine, Japan Specialty section: This article was submitted to Dermatology, a section of the journal

Frontiers in Medicine
INTRODUCTION
BRENTUXIMAB VEDOTIN
IMMUNE CHECKPOINT INHIBITORS
HDAC INHIBITORS
DENILEUKIN DIFTITOX
Findings
AUTHOR CONTRIBUTIONS
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