Open Access

REVIEW

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

Lara Garabedian¹, Sofie Struyf², Ghislain Opdenakker², Silvano Sozzani³, Jo Van Damme², Geneviève Laureys¹

1 Department of Pediatric Hematology and Oncology, University Hospital Ghent, Ghent, Belgium
2 Laboratories of Molecular Immunology and Immunobiology, Rega Institute, University of Leuven (K.U.Leuven), Leuven, Belgium
3 Section of General Pathology and Immunology, University of Brescia, Brescia, Italy

* Corresponding Author: G. Laureys,

European Cytokine Network 2011, 22(3), 148-153. https://doi.org/10.1684/ecn.2011.0290

Accepted 07 September 2011;

Abstract

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

---