What Is New in CTCL—Pathogenesis, Diagnosis, and Treatments
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Purpose of Review
Cutaneous T cell lymphoma (CTCL) is a heterogeneous group of non-Hodgkin’s lymphoma primarily affecting the skin, of which mycosis fungoides (MF) and Sézary syndrome (SS) account for the majority of cases. The pathogenesis of CTCL is poorly understood with no strongly associated environmental or genetic risk factors identified to date. As such, the development of disease-specific therapies has been limited by a lack of understanding of potentially actionable targets. Moreover, the diagnosis of CTCL remains challenging with nonspecific diagnostic criteria, especially in early-stage disease.
The advent of high-throughput sequencing techniques and molecular modalities has provided significant insight into disease pathogenesis, diagnosis, and potential therapies.
Here, we review the classic features of CTCL, focusing on MF/SS, and provide updates on our understanding of this disease.
KeywordsCutaneous T cell lymphoma Mycosis fungoides Sézary syndrome T cell receptor Monoclonal antibody Checkpoint inhibitor
Compliance with Ethical Standards
Conflict of Interest
Dr. Dulmage has a patent “Method for diagnosis, prognosis and determination of treatment for cutaneous t-cell lymphoma” pending.
Drs. Betty Y. Kong, Kassandra Holzem and Joan Guitart declare that they have no conflict of interest.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
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