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

RM Egeler, AGS (Astrid) van Halteren, PCW Hogendoorn, Jon Laman, Pieter Leenen

Research output: Contribution to journalArticleAcademicpeer-review

83 Citations (Scopus)

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.
Original languageUndefined/Unknown
Pages (from-to)213-232
Number of pages20
JournalImmunological Reviews
Volume234
DOIs
Publication statusPublished - 2010

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