VEXAS Syndrome:Report of One Case.
Hui-Ting Liu, Jia-Xin Zhou, Ruo-Xi Zhang et al.
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Case in brief
An elderly male presented with fatigue and fever, later developing macrocytic anemia, tracheal and bronchial wall thickening, polyarthritis, rashes, and elevated inflammatory markers. A bone marrow smear revealed vacuolar degeneration in immature granulocytes, leading to UBA1 gene testing that confirmed VEXAS syndrome. Glucocorticoid therapy improved his condition, but he experienced recurrence of fever and anemia months after treatment cessation.
Diagnostic / clinical pearl
The diagnosis of VEXAS syndrome can be supported by identifying vacuolar degeneration in immature granulocytes on a bone marrow smear, highlighting the importance of integrating clinical and laboratory findings.