Immunopathogenesis and therapy of cutaneous T cell lymphoma.
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.
Kim
Ellen J
EJ
Department of Dermatology, University of Pennsylvania School of Medicine and the Wistar Institute, Philadelphia, Pennsylvania 19104, USA. Ellen.kim@uphs.upenn.edu
Hess
Stephen
S
Richardson
Stephen K
SK
Newton
Sara
S
Showe
Louise C
LC
Benoit
Bernice M
BM
Ubriani
Ravi
R
Vittorio
Carmela C
CC
Junkins-Hopkins
Jacqueline M
JM
Wysocka
Maria
M
Rook
Alain H
AH
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
D015703:Antigens, CD