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ASH 2025 | A new validated staging system for AL amyloidosis: identifying ultra-high-risk disease
Jahanzaib Khwaja, MD, FRCPath, University College London Hospitals NHS Foundation Trust, London, UK, shares insights into a novel staging system for patients with systemic light chain (AL) amyloidosis that aims to identify those with ultra-high-risk disease who may benefit from alternative treatment approaches. This interview took place at the 67th ASH Annual Meeting and Exposition, held in Orlando, FL.
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Transcript
Yeah, so systemic AL Amyloidosis has significantly changed over the last years in terms of outcomes for patients. Traditionally, we’ve used the Mayo 2004, which is modified with the European modification, and then the Mayo 2012 models. And those are really valid and helpful in eras in which we had access to bortezomib and other such agents. We’ve however seen since the introduction of daratumumab outcomes have significantly improved and what was considered as traditionally high risk, so those were 3B and Mayo 4 in the traditional models, now no longer identify the poorest risk disease...
Yeah, so systemic AL Amyloidosis has significantly changed over the last years in terms of outcomes for patients. Traditionally, we’ve used the Mayo 2004, which is modified with the European modification, and then the Mayo 2012 models. And those are really valid and helpful in eras in which we had access to bortezomib and other such agents. We’ve however seen since the introduction of daratumumab outcomes have significantly improved and what was considered as traditionally high risk, so those were 3B and Mayo 4 in the traditional models, now no longer identify the poorest risk disease. So the rationale for developing a new staging model, which is the AL-ISS, was that we want to identify the poor risk patients as well as those who will do well. So 3C identifies the poorest risk. So those are with a longitudinal strain of greater or equal to minus 9%, as well as an NT-proBNP of greater or equal to 8,500 and a troponin greater or equal to 50. And those patients in the modern era perform poorly. We’ve considered those as an ultra-high risk, and we suggest that these patients should be treated with alternate treatment approaches, so consideration of clinical trial design, particularly for these patients, or novel therapies, or consideration in certain selected cases for cardiac transplantation. So I think there’s great relevance for this new prognostic staging model, and I’m hopeful that we’ll use this to shape trial design and our management for these patients in the future.
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