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EBMT 2025 | Challenges in the diagnosis and management of VEXAS syndrome

In this video, Carmelo Gurnari, MD, University of Rome Tor Vergata, Rome, Italy, and Cleveland Clinic, Cleveland, OH, briefly comments on the challenges of VEXAS syndrome, stating that the lack of official guidelines for diagnosis, treatment, and follow-up is a significant obstacle. Dr Gurnari highlights the need for evidence-based guidelines and consensus among experts to provide clear guidance for clinicians managing this disease. This interview took place at the 51st Annual Meeting of the EBMT in Florence, Italy.

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Transcript

First of all, we need to know that for VEXAS we don’t have official guidelines, nor for diagnosis, nor for treatment, nor for follow-up. So the evidence that we have in the literature, and so far the latest PubMed that I did for the meet-the-expert is of April 2025, and a lot of papers have been published, around like 450, regarding VEXAS so we know something that is from evidence, but we don’t have official guidelines, So this is the first challenge – we need official guidelines from an expert and even consensus in order to do the best for these patients, because we need guidance for diagnostics of VEXAS...

First of all, we need to know that for VEXAS we don’t have official guidelines, nor for diagnosis, nor for treatment, nor for follow-up. So the evidence that we have in the literature, and so far the latest PubMed that I did for the meet-the-expert is of April 2025, and a lot of papers have been published, around like 450, regarding VEXAS so we know something that is from evidence, but we don’t have official guidelines, So this is the first challenge – we need official guidelines from an expert and even consensus in order to do the best for these patients, because we need guidance for diagnostics of VEXAS. Peripheral blood versus bone marrow is always in – what is the best source? What is the best technique? And then obviously the therapeutic implication of it. Every patient needs to have MDS screened when is anemic in order to understand if also a potential implication regarding therapeutics can be drawn from this acquired label, acquired diagnostic label. So I will say that in VEXAS it’s a lot to be discovered yet but we have some evidence that we can use to improve our patient’s journey to treatment and to at least control of the disease.

 

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