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J Clin Invest. 2005 Apr;115(4):798-812.

Immunopathogenesis and therapy of cutaneous T cell lymphoma.

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  • 1Department of Dermatology, University of Pennsylvania School of Medicine and the Wistar Institute, Philadelphia, Pennsylvania 19104, USA.

Erratum in

  • J Clin Invest. 2007 Mar;117(3):836.


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.

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