期刊
LEUKEMIA
卷 26, 期 3, 页码 424-432出版社
NATURE PUBLISHING GROUP
DOI: 10.1038/leu.2011.237
关键词
cutaneous T-cell lymphoma; Sezary syndrome; immunodeficiency; regulatory T cells; FOXP3; mycosis fungoides
资金
- Carlsberg Foundation (Carlsbergfondet)
- Danish Research Councils
- Danish Cancer Society
- Lundbeck Foundation
- Novo Nordic Foundation
- Fabrikant Vilhelm Pedersen og Hustrus Mindelegat
- Neye Foundation
- University of Copenhagen
- National Cancer Institute [CA89194]
Cutaneous T-cell lymphoma (CTCL) is the term for diseases characterized by primary accumulation of malignant T cells in the skin. Patients with the two predominant clinical forms of CTCL called mycosis fungoides (MF) and Sezary syndrome (SS) characteristically develop severe immunodeficiency during disease progression and consequently patients with advanced disease frequently die of infections and not from the tumor burden. For decades, it has been suspected that the malignant T cells actively drive the evolving immunodeficiency to avoid antitumor immunity, yet, the underlying mechanisms remain unclear. The identification of a subset of highly immunosuppressive regulatory T cells (Tregs) triggered a variety of studies investigating if MF and SS are malignant proliferations of Tregs but seemingly discordant findings have been reported. Here, we review the literature to clarify the role of Tregs in MF and SS and discuss the potential mechanisms driving the immunodeficiency. Leukemia (2012) 26, 424-432; doi:10.1038/leu.2011.237; published online 9 September 2011
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