1. Case Report: Evolution of a Severe Vascular Refractory Form of ECD Requiring Liver Transplantation Correlated With the Change in the Monocyte Subset Analysis.
- Author
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Razanamahery J, Roggy A, Emile JF, Malakhia A, Lakkis Z, Garnache-Ottou F, Soumagne T, Cohen-Aubart F, Haroche J, and Bonnotte B
- Subjects
- Adult, Erdheim-Chester Disease complications, Erdheim-Chester Disease immunology, Fatal Outcome, Female, Humans, Immunophenotyping, Liver Failure, Acute pathology, Liver Failure, Acute surgery, Erdheim-Chester Disease pathology, Liver Failure, Acute etiology, Liver Transplantation, Monocytes immunology, Monocytes pathology
- 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 CD14low 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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Razanamahery, Roggy, Emile, Malakhia, Lakkis, Garnache-Ottou, Soumagne, Cohen-Aubart, Haroche and Bonnotte.)- Published
- 2021
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