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

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...

Full description

Bibliographic Details
Main Authors: Frederik Staels, Albrecht Betrains, F. J. Sherida H. Woei-A-Jin, Nancy Boeckx, Marielle Beckers, An Bervoets, Mathijs Willemsen, Barbara Neerinckx, Stephanie Humblet-Baron, Daniel Engelbert Blockmans, Steven Vanderschueren, Rik Schrijvers
Format: Article
Language:English
Published: Frontiers Media S.A. 2021-04-01
Series:Frontiers in Immunology
Subjects:
primary immunodeficiencies
autoinflammation
treatment reactions
myelodysplasia
ubiquitination
Online Access:https://www.frontiersin.org/articles/10.3389/fimmu.2021.678927/full

Internet

https://www.frontiersin.org/articles/10.3389/fimmu.2021.678927/full

Similar Items