title: Immunopathogenesis and therapy of cutaneous T cell lymphoma.
abstract: Cutaneous T cell lymphomas (CTCLs) are a heterogenous group of lymphoproliferative disorders caused by clonally derived, skin-invasive T cells. Mycosis fungoides (MF) and Sezary syndrome (SS) are the most common types of CTCLs and are characterized by malignant CD4(+)/CLA(+)/CCR4(+) T cells that also lack the usual T cell surface markers CD7 and/or CD26. As MF/SS advances, the clonal dominance of the malignant cells results in the expression of predominantly Th2 cytokines, progressive immune dysregulation in patients, and further tumor cell growth. This review summarizes recent insights into the pathogenesis and immunobiology of MF/SS and how these have shaped current therapeutic approaches, in particular the growing emphasis on enhancement of host antitumor immune responses as the key to successful therapy.
authors.affiliation: Department of Dermatology, University of Pennsylvania School of Medicine and the Wistar Institute, Philadelphia, Pennsylvania 19104, USA. Ellen.kim@uphs.upenn.edu
authors.forename: Ellen J
authors.initials: EJ
authors.lastname: Kim
authors.forename: Stephen
authors.initials: S
authors.lastname: Hess
authors.forename: Stephen K
authors.initials: SK
authors.lastname: Richardson
authors.forename: Sara
authors.initials: S
authors.lastname: Newton
authors.forename: Louise C
authors.initials: LC
authors.lastname: Showe
authors.forename: Bernice M
authors.initials: BM
authors.lastname: Benoit
authors.forename: Ravi
authors.initials: R
authors.lastname: Ubriani
authors.forename: Carmela C
authors.initials: CC
authors.lastname: Vittorio
authors.forename: Jacqueline M
authors.initials: JM
authors.lastname: Junkins-Hopkins
authors.forename: Maria
authors.initials: M
authors.lastname: Wysocka
authors.forename: Alain H
authors.initials: AH
authors.lastname: Rook
mesh_terms: D015703:Antigens, CD / Q000276:immunology; D006801:Humans; D007167:Immunotherapy / Q000379:methods; D016410:Lymphoma, T-Cell, Cutaneous / Q000209:etiology / Q000276:immunology / Q000473:pathology / Q000628:therapy; D009367:Neoplasm Staging; D012867:Skin / Q000166:cytology / Q000473:pathology; D012878:Skin Neoplasms / Q000209:etiology / Q000276:immunology / Q000473:pathology / Q000628:therapy; D015996:Survival Rate; D016176:T-Lymphocyte Subsets / Q000276:immunology; D013601:T-Lymphocytes / Q000276:immunology
chemical_list: D015703:Antigens, CD