Article
Erdheim-Chester disease: An in vivo human model of Mϕ activation at the crossroad between chronic inflammation and cancer.
Journal of leukocyte biology - 1 Aug 2020
Cavalli Giulio, Dagna Lorenzo, Biavasco Riccardo, Villa Antonello, Doglioni Claudio, Ferrero Elisabetta, Ferrarini Marina
Abstract excerpt
Erdheim-Chester disease (ECD) is a rare histiocytosis characterized by infiltration of multiple tissues by CD68+ foamy Mϕs (or 'histiocytes'). Clinical manifestations arise from mass-forming lesions or from tissue and systemic inflammation. ECD histiocytes harbor oncogenic mutations along the MAPK-kinase signaling pathway (BRAFV600E in more than half of the patients), and secrete abundant pro-inflammatory...
Topics
- Animals
- Cellular Reprogramming
- Disease Susceptibility
- Energy Metabolism
- Erdheim-Chester Disease
- Histiocytes
- Humans
- Inflammation
- Macrophage Activation
- Macrophages
- Mutation
- Neoplasms
- Oncogenes
- Signal Transduction