What is VEXAS syndrome: A syndrome with a mix of inflammation and hematologic disorder.
Often associated with MDS Or MGUS.
UBA1 gene inactivating mutations
Most effective rx so far to date: Jakafi ( Blood Aug 2022)
Adult-onset syndrome that links seemingly disparate hematologic and autoimmune symptoms. The first description of the disorder, called VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome, was published in The New England Journal of Medicine (NEJM) in December 2020
https://www.nejm.org/doi/full/10.1056/NEJMoa2026834
https://ashpublications.org/ashclinicalnews/news/5960/The-Vexing-VEXAS-Syndrome?searchresult=1
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