Somatic mutation in the gene encoding for UBA1 has been recently described in patients with adult-onset inflammatory diseases, defining a novel disease called VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome which is frequently associated with MDS (25 to 50% of VEXAS cases) [84,85]. This evidence concerns the gene UBA1 and VEXAS syndrome.