Pathological Consequence of Misguided Dendritic Cell Differentiation in Histiocytic Diseases

  • Marie Luise Berres
  • , Carl E. Allen
  • , Miriam Merad

Research output: Chapter in Book/Report/Conference proceedingChapterpeer-review

70 Scopus citations

Abstract

Histiocytic disorders represent a group of complex pathologies characterized by the accumulation of histiocytes, an old term for tissue-resident macrophages and dendritic cells. Langerhans cell histiocytosis is the most frequent of histiocytosis in humans and has been thought to arise from the abnormal accumulation of epidermal dendritic cells called Langerhans cells. In this chapter, we discuss the origin and differentiation of Langerhans cells and dendritic cells and present accumulated evidence that suggests that Langerhans cell histiocytosis does not result from abnormal Langerhans cell homeostasis but rather is a consequence of misguided differentiation programs of myeloid dendritic cell precursors. We propose reclassification of Langerhans cell histiocytosis, juvenile xanthogranuloma, and Erdheim-Chester disease as inflammatory myeloid neoplasias.

Original languageEnglish
Title of host publicationAdvances in Immunology
PublisherAcademic Press Inc.
Pages127-161
Number of pages35
DOIs
StatePublished - 2013

Publication series

NameAdvances in Immunology
Volume120
ISSN (Print)0065-2776
ISSN (Electronic)1557-8445

Keywords

  • BRAFV600E
  • DC differentiation
  • Dendritic cell lineage
  • Erdheim-Chester disease
  • Histiocytic disorders
  • Juvenile xanthogranuloma
  • Langerhans cell histiocytosis
  • Macrophages

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