UBA1 and VEXAS syndrome: In late 2020, Beck et al. described somatic loss of function variants in UBA1 in the hematopoietic stem and progenitor cell (HSPC) compartment as a cause of a severe hematoinflammatory disease termed VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) Syndrome [4].