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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Udgivet i:Front Immunol
Main Authors: 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
Format: Artigo
Sprog:Inglês
Udgivet: Frontiers Media S.A. 2021
Fag:
Immunology
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC8147557/
https://ncbi.nlm.nih.gov/pubmed/34046042
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3389/fimmu.2021.678927
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