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Case Report: Evolution of a Severe Vascular Refractory Form of ECD Requiring Liver Transplantation Correlated With the Change in the Monocyte Subset Analysis
- Source :
- Frontiers in Immunology, Frontiers in Immunology, Frontiers, 2021, 12, ⟨10.3389/fimmu.2021.755846⟩, Frontiers in Immunology, Vol 12 (2021)
- Publication Year :
- 2021
- Publisher :
- HAL CCSD, 2021.
-
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.
- Subjects :
- Pathology
medicine.medical_specialty
Myeloid
CD14
Immunology
Erdheim–Chester disease
Case Report
CD16
Pathogenesis
03 medical and health sciences
0302 clinical medicine
medicine
Immunology and Allergy
030304 developmental biology
0303 health sciences
business.industry
Monocyte
RC581-607
medicine.disease
3. Good health
Transplantation
Histiocytosis
Vascular diagnosis
medicine.anatomical_structure
030220 oncology & carcinogenesis
monocyte
Erdheim-Chester disease
Immunologic diseases. Allergy
business
histiocytosis
[SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
transplantation
Subjects
Details
- Language :
- English
- ISSN :
- 16643224
- Database :
- OpenAIRE
- Journal :
- Frontiers in Immunology, Frontiers in Immunology, Frontiers, 2021, 12, ⟨10.3389/fimmu.2021.755846⟩, Frontiers in Immunology, Vol 12 (2021)
- Accession number :
- edsair.doi.dedup.....ab79ce992877ef91f2123578b9e2d0c2