Department of Internal Medicine and Clinical Immunology, Francois Mitterrand Hospital, Dijon University Hospital, Dijon, France.
Établissement Français du Sang Bourgogne Franche-Comté, Laboratoire d'Hématologie et d'Immunologie Régional, Besançon, France.
Front Immunol. 2021 Nov 12;12:755846. doi: 10.3389/fimmu.2021.755846. eCollection 2021.
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 CD14CD16 "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 CD14CD16 "classical monocyte" associated with decreased CD14CD16 "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.
厄尔-道伊姆-切斯特病是一种罕见的组织细胞增生症，其特征为具有与相容组织学相关的标志性特征。大多数患者存在 MAP 激酶通路基因的体细胞突变，这些突变发生在 CD14 单核细胞中。髓系分化在组织细胞增生症的发病机制中起着核心作用。单核细胞是髓系来源的白细胞，分为三个亚群，但只有 CD14CD16“经典单核细胞”可以分化为树突状细胞和组织巨噬细胞。由于大多数突变发生在 CD14 细胞中，并且由于 ECD 患者具有类似于 CMML 的特定单核细胞表型，因此我们研究了在需要进行肝移植的严重血管形式的 ECD 病程中，疾病活动与单核细胞亚群分布之间的相关性。在早期随访中，与疾病活动相关的是 CD14CD16“经典单核细胞”增加，而 CD14CD16“非经典单核细胞”减少。需要进一步的研究来证实单核细胞作为 ECD 患者疾病活动的标志物的用途。
