Multidisciplinary Management of Mycosis Fungoides/Sézary Syndrome

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) a...

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Bibliographic Details
Published in:Current hematologic malignancy reports 2017-06, Vol.12 (3), p.234-243
Main Authors: Berg, Sara, Villasenor-Park, Jennifer, Haun, Paul, Kim, Ellen J.
Format: Article
Language:English
Subjects:
B-cell NHL
Biomarkers, Tumor
Combined Modality Therapy
Disease Management
Genomics - methods
Geriatrics/Gerontology
Hematology
Hodgkin Lymphoma
Hodgkin Lymphoma (D Persky
Humans
Medicine
Medicine & Public Health
Mycosis Fungoides - diagnosis
Mycosis Fungoides - etiology
Mycosis Fungoides - therapy
Oncology
Prognosis
Section Editor
Sezary Syndrome - diagnosis
Sezary Syndrome - etiology
Sezary Syndrome - therapy
Skin Neoplasms - diagnosis
Skin Neoplasms - etiology
Skin Neoplasms - therapy
T-cell NHL
Topical Collection on B-cell NHL
Treatment Outcome
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Summary: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.
ISSN:1558-8211
1558-822X
DOI:10.1007/s11899-017-0387-9