In:
Frontiers in Immunology, Frontiers Media SA, Vol. 12 ( 2021-11-12)
Kurzfassung:
Erdheim–Chester disease is a rare histiocytosis characterized by iconic features associated with compatible histology. Most patients have somatic mutations in the MAP-kinase pathway gene, and the mutations occur in CD14 + monocytes. Differentiation of the myeloid lineage plays a central role in the pathogenesis of histiocytosis. Monocytes are myeloid-derived white blood cells, divided into three subsets, but only the CD14 ++ CD16 − “classical monocyte” can differentiate into dendritic cells and tissue macrophages. Since most mutations occur in CD14 + cells and since ECD patients have a particular monocytic phenotype resembling CMML, we studied the correlation between disease activity and monocytic subset distribution during the course of a severe vascular form of ECD requiring liver transplantation. During early follow-up, increased CD14 ++ CD16 − “classical monocyte” associated with decreased CD14 low CD16 ++ “non-classical monocyte” correlated with disease activity. Further studies are needed to confirm the use of monocyte as a marker of disease activity in patients with ECD.
Materialart:
Online-Ressource
ISSN:
1664-3224
DOI:
10.3389/fimmu.2021.755846
Sprache:
Unbekannt
Verlag:
Frontiers Media SA
Publikationsdatum:
2021
ZDB Id:
2606827-8