LAUSR

A 70-year-old Chinese male presented with unexplained fever, relapsing polychondritis, macrocytic anemia, acute necrotising lymphadenitis, skin papules that were in keeping histologically with Kikuchi-Fujimoto disease, and pulmonary embolism secondary to extensive unprovoked right lower limb deep vein thrombosis. Given the recent discovery of VEXAS (vacuoles, E1 enzyme, X-linked, IMAGE 1 Vacuolation seen in the haematopoietic cells from the bone marrow aspirate of the patient with VEXAS syndrome, with accompanying features of trilineage dysplasia. Top left (A): Vacuolation in a myelomonocytic precursor cell. Top center left (B): Vacuolation in a monocytic precursor cell. Top center right (C): Vacuolation in a myelomonocytic precursor cell. Top right (D): Vacuolation in an erythroblast. Bottom left (E), Bottom center left (F): Vacuolation in giant platelets. Bottom center right (G), Bottom right (H): Vacuolation in plasma cells Received: 24 December 2020 Revised: 12 January 2021 Accepted: 14 January 2021