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Multidisciplinary Management of Mycosis Fungoides/Sézary Syndrome

  • B-cell NHL, T-cell NHL, and Hodgkin Lymphoma (D Persky, Section Editor)
  • Published:
Current Hematologic Malignancy Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

Diagnosis and management of mycosis fungoides and Sézary syndrome (MF/SS) require accurate clinicopathological correlation and a multidisciplinary approach. We reviewed major advances in the field regarding diagnostic and prognostic tools as well as skin-directed therapies (SDTs) and systemic agents for MF/SS published in the past 2 years.

Recent Findings

Improved technology (T-cell receptor high-throughput sequencing) and increased multicenter collaboration (Cutaneous Lymphoma International Consortium) have led to diagnostic/prognostic advances. Concurrently, numerous genomic studies have enhanced understanding of disease pathogenesis. Advances in SDTs include topical resiquimod, a novel potent Toll-like receptor (TLR) agonist; consensus CTCL phototherapy guidelines; and use of low-dose radiation therapy. Novel systemic therapies for advanced disease of note include targeted antibody drug conjugates (brentuximab vedotin), immune checkpoint inhibitors, and allogeneic hematopoietic stem cell transplantation (HSCT).

Summary

Our “toolbox” to diagnose and treat the spectrum of MF/SS continues to expand. Further characterization of genomic data going forward will enable a rational approach to selecting and combining therapies to improve patient care.

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Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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  1. Penn Cutaneous Lymphoma Program, Department of Dermatology, Perelman Center for Advanced Medicine, Perelman School of Medicine at the University of Pennsylvania, 1st Floor South Pavilion, 3400 Civic Center Boulevard, Philadelphia, PA, 19104, USA

    Sara Berg, Jennifer Villasenor-Park, Paul Haun & Ellen J. Kim

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Sara Berg and Jennifer Villasenor-Park each declare no potential conflicts of interest. Paul Haun reports royalties from Health Press, Inc., Fast Facts: Diagnosing Cutaneous T-cell Lymphoma. Ellen J. Kim reports grants from Actelion, Kyowa Kirin, and Solgenix.

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This article is part of the Topical Collection on B-cell NHL, T-cell NHL, and Hodgkin Lymphoma

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Berg, S., Villasenor-Park, J., Haun, P. et al. Multidisciplinary Management of Mycosis Fungoides/Sézary Syndrome. Curr Hematol Malig Rep 12, 234–243 (2017). https://doi.org/10.1007/s11899-017-0387-9

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