Cutaneous T-Cell Lymphoma: A Review with a Focus on Targeted Agents

Am J Clin Dermatol. 2016 Jun;17(3):225-37. doi: 10.1007/s40257-016-0177-5.

Abstract

Cutaneous T-cell lymphomas (CTCLs) are a heterogeneous group of extranodal lymphomas involving the skin. Diagnosis of the two main subtypes of CTCL-mycosis fungoides (MF) and Sézary syndrome (SS)-is based on the International Society for Cutaneous Lymphomas/European Organization for Research and Treatment of Cancer (ISCL/EORTC) classification system, which utilizes clinical, histopathological, molecular biologic, and immunopathologic features. Risk stratification, based on TNMB (tumor, node, metastasis, and blood) staging, provides prognostic information, with limited-stage disease conferring the longest median overall survival. Skin-directed therapies are preferred in the management of limited-stage disease, whereas advanced-stage disease requires systemic therapies. As the mechanisms of CTCL pathogenesis are increasingly understood, new monoclonal antibodies, checkpoint inhibitors, immunomodulatory agents, and small molecules are under investigation and may provide additional therapeutic options for those with advanced CTCL. This review examines the current landscape of targeted therapies in the treatment of CTCLs.

Publication types

  • Review

MeSH terms

  • Antibodies, Monoclonal, Humanized / administration & dosage
  • Antibodies, Monoclonal, Humanized / adverse effects
  • Antibodies, Monoclonal, Humanized / therapeutic use
  • Antineoplastic Agents / administration & dosage
  • Antineoplastic Agents / adverse effects
  • Antineoplastic Agents / therapeutic use*
  • Cell Proliferation
  • Clinical Trials as Topic
  • Combined Modality Therapy
  • Epigenesis, Genetic
  • Histone Deacetylase Inhibitors / administration & dosage
  • Histone Deacetylase Inhibitors / adverse effects
  • Histone Deacetylase Inhibitors / therapeutic use
  • Humans
  • Immunologic Factors / administration & dosage
  • Immunologic Factors / adverse effects
  • Immunologic Factors / therapeutic use
  • Molecular Targeted Therapy / methods*
  • Mycosis Fungoides / drug therapy*
  • Mycosis Fungoides / etiology
  • Mycosis Fungoides / pathology
  • Neoplasm Staging
  • Phototherapy / methods
  • Receptors, Antigen, T-Cell / genetics
  • Sezary Syndrome / drug therapy*
  • Sezary Syndrome / etiology
  • Sezary Syndrome / pathology
  • Signal Transduction
  • Skin / pathology
  • Skin Neoplasms / drug therapy*
  • Skin Neoplasms / etiology
  • Skin Neoplasms / pathology
  • T-Lymphocytes / physiology
  • Tumor Microenvironment

Substances

  • Antibodies, Monoclonal, Humanized
  • Antineoplastic Agents
  • Histone Deacetylase Inhibitors
  • Immunologic Factors
  • Receptors, Antigen, T-Cell