VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome has been recently described as a severe autoinflammatory disease associated with hematological manifestations such as vacuolization of precursors and macrocytosis.1,2 VEXAS is the consequence of somatically acquired mutations of ubiquitin-like modifier activating enzyme 1 (UBA1), which impair the translation of the cytoplasmic isoform of the enzyme (UBA1b). Here, UBA1 is linked to VEXAS syndrome.