[Chronic anemia and unexplained inflammation: think of VEXAS syndrome].

Paul L A van Daele,Caspar I van der Made,Helen L Leavis,A E Liesbeth Hak,Judith Potjewijd,Abraham Bram Rutgers

Nederlands tijdschrift voor geneeskunde（2023）

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摘要

In middle aged males presenting with multisystemic inflammation without evidence of infection a diagnosis of VEXAS should be considered, especially if there is evidence of a macrocytic anemia. Early testing for UBA1 mutations helps in making the diagnosis. Despite treatment with intensive immunosuppression mortality remains high.

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