Article
Unexplained fever with consumptive syndrome in the elderly: two cases of VEXAS syndrome with inflammasome dysregulation.
Clinical and experimental immunology - 16 Oct 2024
Mendonça Leonardo Oliveira, Leal Vinicius N C, Roa Mariela E G V, Barros Samar Freschi, Kalil Jorge, Pontillo Alessandra
Abstract excerpt
The aim of this study is to investigate the inflammasome dysregulation in peripheral blood leukocytes of VEXAS patients. The constitutive and in vitro triggered activation of inflammasome in PBMC and neutrophils was analyzed in two Brazilian patients with typical UBA1 mutations, and compared with healthy donors. Our findings highlight the constitutive activation of caspase-1 in VEXAS leukocytes, accompanied by...
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