Langerhans cell histiocytosis: fascinating dynamics of the dendritic cell-macrophage lineage

Department of Pediatric Immunology, Hematology, Oncology, Bone Marrow Transplantation and Autoimmune Diseases, Leiden University Medical Center (LUMC), Leiden, The Netherlands.
Immunological Reviews (Impact Factor: 12.91). 03/2010; 234(1):213-32. DOI: 10.1111/j.0105-2896.2009.00883.x
Source: PubMed

ABSTRACT In its rare occurrence, Langerhans cell histiocytosis (LCH) is a dangerous but intriguing deviation of mononuclear phagocytes, especially dendritic cells (DCs). Clinically, the disease ranges from self-resolving or well manageable to severe and even fatal. LCH lesions in skin, bone, and other sites contain high numbers of cells with phenotypic features resembling LCs admixed with macrophages, T cells, eosinophils, and multinucleated giant cells. Here we review current progress in the LCH field based on two central questions: (i) are LCH cells intrinsically aberrant, and (ii) how does the lesion drive pathogenesis? We argue that LCH cells may originate from different sources, including epidermal LCs, tissue Langerin(+) DCs, or mononuclear phagocyte precursors. Current and prospective in vitro and in vivo models are discussed. Finally, we discuss recent insights into plasticity of T-helper cell subsets in light of the lesion microenvironment. LCH continues to provide urgent clinical questions thereby inspiring innovative DC lineage research.


Available from: Pancras C W Hogendoorn, May 29, 2015
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