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Langerhans cell histiocytosis: a cytokine/chemokine-mediated disorder?


European Cytokine Network. Volume 22, Number 3, 148-53, September 2011, Review article

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Author(s) : Lara Garabedian, Sofie Struyf, Ghislain Opdenakker, Silvano Sozzani, Jo Van Damme, Geneviève Laureys

Summary : Langerhans cell histiocytosis (LCH) is a rare disorder characterized by an abnormal accumulation and/or proliferation of cells with a Langerhans cell phenotype. Although no clear cause of LCH has been identified, it has been postulated that LCH might be the consequence of an immune dysregulation, causing Langerhans cells to migrate to and accumulate at various sites. Production of cytokines and chemokines is a central feature of immune regulation. Cytokines are abundantly present within LCH lesions. We review here the potential role of cytokines and chemokines in the pathogenesis of LCH. The type, distribution, and number of different cytokines released within lesions can provide clues to the possible aetiology of LCH and, ultimately, might offer therapeutic possibilities using recombinant cytokines or antagonists for this disorder.

Keywords : Langerhans cell histiocytosis, cytokines, chemokines, dendritic cells

 

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