Sara Berg1, Jennifer Villasenor-Park1, Paul Haun1, Ellen J Kim2. 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. 2. 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. ellen.kim@uphs.upenn.edu.
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). 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.
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). 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.
Entities:
Keywords:
Consensus CTCL phototherapy; Cutaneous Lymphoma International Consortium; MF/SS; Mycosis fungoides and Sézary syndrome; Toll-like receptor agonist
Authors: Youn H Kim; Mahkam Tavallaee; Uma Sundram; Katrin A Salva; Gary S Wood; Shufeng Li; Sima Rozati; Seema Nagpal; Michael Krathen; Sunil Reddy; Richard T Hoppe; Annie Nguyen-Lin; Wen-Kai Weng; Randall Armstrong; Melissa Pulitzer; Ranjana H Advani; Steven M Horwitz Journal: J Clin Oncol Date: 2015-07-20 Impact factor: 44.544
Authors: Emmanuelle Tancrède-Bohin; Marius Anton Ionescu; Pauline de La Salmonière; Alain Dupuy; Jacqueline Rivet; Michel Rybojad; Louis Dubertret; Hervé Bachelez; Celeste Lebbé; Patrice Morel Journal: Arch Dermatol Date: 2004-09
Authors: E C Benton; S Crichton; R Talpur; N S Agar; P A Fields; E Wedgeworth; T J Mitchell; M Cox; S Ferreira; P Liu; A Robson; E Calonje; C M Stefanato; B Wilkins; J Scarisbrick; E M Wain; F Child; S Morris; M Duvic; S J Whittaker Journal: Eur J Cancer Date: 2013-06-01 Impact factor: 9.162
Authors: Linghua Wang; Xiao Ni; Kyle R Covington; Betty Y Yang; Jessica Shiu; Xiang Zhang; Liu Xi; Qingchang Meng; Timothy Langridge; Jennifer Drummond; Lawrence A Donehower; Harshavardhan Doddapaneni; Donna M Muzny; Richard A Gibbs; David A Wheeler; Madeleine Duvic Journal: Nat Genet Date: 2015-11-09 Impact factor: 38.330
Authors: Andreas Willerslev-Olsen; Terkild B Buus; Claudia Nastasi; Edda Blümel; Maria Gluud; Charlotte M Bonefeld; Carsten Geisler; Lise M Lindahl; Maarten Vermeer; Mariusz A Wasik; Lars Iversen; Jürgen C Becker; Mads Hald Andersen; Lise M R Gjerdrum; Ivan V Litvinov; Thomas Litman; Thorbjørn Krejsgaard; Anders Woetmann; Niels Ødum Journal: Blood Cancer J Date: 2020-05-14 Impact factor: 11.037