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EHA 2026 | Advances in the clinical understanding of advanced SM and educational gaps that remain
Deepti Radia, MBBS, BSc, MRCPI, FRCPath, MSc Med Ed, Guy’s and St Thomas’ NHS Foundation Trust, London, UK, briefly discusses advances in the clinical understanding of advanced systemic mastocytosis (SM), highlighting the impact of improved knowledge on the molecular landscape of the disease on risk stratification and prognostication. She also notes that while significant progress has been made, much remains to be learned about the associated hematologic neoplasm (AHN) component of advanced SM, which is present in the majority of patients. This interview took place at the 31st Congress of the European Hematology Association (EHA) in Stockholm, Sweden.
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Transcript
So what’s changed in the last 10 years apart from having TKIs that have taught us a lot in trials? The molecular landscape. So looking at the advanced SM, we know that the majority of patients, 70% have SM and an AHN. We are now looking at the more somatic mutations in addition to CKIT and the SAR panel. So the new exciting data is on the MARS-R, which is a new prognostic group developed by the Mannheim group with Johannes Lupe leading on it...
So what’s changed in the last 10 years apart from having TKIs that have taught us a lot in trials? The molecular landscape. So looking at the advanced SM, we know that the majority of patients, 70% have SM and an AHN. We are now looking at the more somatic mutations in addition to CKIT and the SAR panel. So the new exciting data is on the MARS-R, which is a new prognostic group developed by the Mannheim group with Johannes Lupe leading on it. And that’s been validated in the TKI era, which is great. So there is now a QRS code and you can apply it, and it actually risk stratifies the patient into low risk, high risk, and intermediate risk, really great with helping past having made the diagnosis, where you might take these patients to treatment, how they might respond to TKI, and potentially guiding which patients might be amenable or move forward to transplant a bit better. Hasn’t changed the fact that we’re still learning about the AHN, the progression of the AHN, so there’s still a lot more to learn. But the use of the prognostic scoring, especially those in the TKI era, are going to be really useful moving us forward.
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