Case Report: Evolution of a Severe Vascular Refractory Form of ECD Requiring Liver Transplantation Correlated With the Change in the Monocyte Subset Analysis.
Abstract
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 CD14lowCD16++ "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.
Authors: | Razanamahery J, Roggy A, Emile JF, Malakhia A, Lakkis Z, Garnache-Ottou F, Soumagne T, Cohen-Aubart F, Haroche J, Bonnotte B. |
---|---|
Journal: | Front Immunol. 2021 Nov 12;12:755846. doi: 10.3389/fimmu.2021.755846. |
Year: | 2021 |
PubMed: | PMID: 34867991 (Go to PubMed) |