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Case Report: VEXAS Syndrome: From Mild Symptoms to Life-Threatening Macrophage Activation Syndrome

Recently, a novel disorder coined VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome was identified in patients with adult-onset inflammatory syndromes, often accompanied by myelodysplastic syndrome1. All patients had myeloid lineage-restricted somatic mutations in UBA1 affect...

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Detalles Bibliográficos
Autores principales: Staels, Frederik, Betrains, Albrecht, Woei-A-Jin, F. J. Sherida H., Boeckx, Nancy, Beckers, Marielle, Bervoets, An, Willemsen, Mathijs, Neerinckx, Barbara, Humblet-Baron, Stephanie, Blockmans, Daniel Engelbert, Vanderschueren, Steven, Schrijvers, Rik
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8147557/
https://www.ncbi.nlm.nih.gov/pubmed/34046042
http://dx.doi.org/10.3389/fimmu.2021.678927