Somatic mutations in nonhematopoietic genes recently identified in human patients with adult-onset severe autoinflammatory disease and MDS, such as the VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic UBA1 mutations) syndrome, link autoimmunity and hematopoietic disease, and further highlight the complexity of diagnosis and classification of myeloid neoplasia.19 This evidence concerns the gene UBA1 and Autoimmunity.