Search

Keywords: vacuoles; myelodysplastic syndromes; ubiquitin-activating enzymes; VEXAS syndrome; autoinflammatory disorder EN vacuoles myelodysplastic syndromes ubiquitin-activating enzymes VEXAS syndrome autoinflammatory disorder 286 289 4 10/12/21 20211015 NES 211015 VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a recently described adult-onset inflammatory disease,1 responsible for inflammatory and haematological disorders.2 The presence of bone marrow vacuoles appeared to be greatly significant in the diagnosis of VEXAS syndrome.1 However, myeloid and erythroid vacuolization has been described in other disorders: copper deficiency,3 acute myeloid leukaemia,4 Menkès disease5 and transcobalamin II deficiency.6 Nevertheless, these disorders are not associated with chronic inflammatory signs. Vacuoles, myelodysplastic syndromes, ubiquitin-activating enzymes, autoinflammatory disorder, VEXAS syndrome Vacuoles in neutrophil precursor cells in bone marrow analysis of a patient in the VEXAS group (A) and a patient in the MDS group (B).Table SI. [Extracted from the article]