title: Evolving insights in the pathogenesis and therapy of cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome).
abstract: Cutaneous T-cell lymphomas (CTCL) are a heterogeneous group of malignancies derived from skin-homing T cells. The most common forms of CTCL are Mycosis Fungoides (MF) and Sezary Syndrome (SS). Accurate diagnosis remains a challenge due to the heterogeneity of presentation and the lack of highly characteristic immunophenotypical and genetic markers. Over the past decade molecular studies have improved our understanding of the biology of CTCL. The identification of gene expression differences between normal and malignant T-cells has led to promising new diagnostic and prognostic biomarkers that now need validation to be incorporated into clinical practice. These biomarkers may also provide insight into the mechanism of development of CTCL. Additionally, treatment options have expanded with the approval of new agents, such as histone deacetylase inhibitors. A better understanding of the cell biology, immunology and genetics underlying the development and progression of CTCL will allow the design of more rational treatment strategies for these malignancies. This review summarizes the clinical epidemiology, staging and natural history of MF and SS; discusses the immunopathogenesis of MF and the functional role of the malignant T-cells; and reviews the latest advances in MF and SS treatment.
authors: Division of Dermatology, The Ohio State University, Columbus, OH 43221, USA. henry.wong@osumc.edu
authors: Henry K
authors: HK
authors: Wong
authors: Anjali
authors: A
authors: Mishra
authors: Timothy
authors: T
authors: Hake
authors: Pierluigi
authors: P
authors: Porcu
mesh_terms: D000818:Animals; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D000951:Antigens, Neoplasm / Q000032:analysis; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis; D002454:Cell Differentiation; D002471:Cell Transformation, Neoplastic; D002869:Chromosome Aberrations; D002986:Clinical Trials as Topic / Q000706:statistics & numerical data; D016207:Cytokines / Q000378:metabolism; D018450:Disease Progression; D052138:Genes, Neoplasm; D018380:Hematopoietic Stem Cell Transplantation; D056572:Histone Deacetylase Inhibitors / Q000627:therapeutic use; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D007157:Immunologic Surveillance; D007167:Immunotherapy / Q000379:methods*; D051379:Mice; D015337:Multicenter Studies as Topic / Q000706:statistics & numerical data; D009182:Mycosis Fungoides / Q000453:epidemiology / Q000209:etiology* / Q000235:genetics / Q000276:immunology / Q000628:therapy*; D012751:Sezary Syndrome / Q000453:epidemiology / Q000209:etiology* / Q000235:genetics / Q000276:immunology / Q000628:therapy*; D012878:Skin Neoplasms / Q000453:epidemiology / Q000209:etiology* / Q000235:genetics / Q000276:immunology / Q000628:therapy*; D016176:T-Lymphocyte Subsets / Q000378:metabolism / Q000473:pathology
chemical_list: D000911:Antibodies, Monoclonal; D000951:Antigens, Neoplasm; D014408:Biomarkers, Tumor; D016207:Cytokines; D056572:Histone Deacetylase Inhibitors; D007155:Immunologic Factors