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COMy 2020 | The importance of early diagnosis for improved light chain amyloidosis survival

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Giampaolo Merlini

Giampaolo Merlini, MD, Foundation IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy, discusses the importance of an early diagnosis for treating light chain amyloidosis. Most patients present with an advanced stage of the disease with a very high concentration of protein. The survival of these patients is very short. However, with early diagnosis the disease is treatable. Early diagnosis can be achieved through the use of new biomarkers and improving awareness of the disease. This interview took place during the Controversies in Multiple Myeloma (COMy) 2020 Virtual World Congress.

Transcript (edited for clarity)

Well, the challenge is now that we have such powerful therapies, is just to identify these patients in the early phase of the disease, because we know that even with powerful therapy, still there is a proportion of about 20% of patients who present with very advanced cardiac involvement with NT-proBNP above 8,500 nanogram per liter, and these patients unfortunately, their prognosis of just few weeks...

Well, the challenge is now that we have such powerful therapies, is just to identify these patients in the early phase of the disease, because we know that even with powerful therapy, still there is a proportion of about 20% of patients who present with very advanced cardiac involvement with NT-proBNP above 8,500 nanogram per liter, and these patients unfortunately, their prognosis of just few weeks. So we have to reduce as much as possible this population through early diagnosis, and early diagnosis, in my view, is the most important goal now in our field.

We have to identify this patient in time. We can do this using biomarkers, NT-proBNP, and albumin in urine in patient using the follow-up for MGUS, but … and also alkaline phosphatase, but also using imaging, that now may be extremely useful to detect from echo to magnetic resonance to scintigraphy with tracer used in Alzheimer’s disease, and most importantly; using bone tracer to detect cardiac amyloid due to TTR; Transthyretin amyloidosis.

There are also new biomarkers focusing on the characteristic of the light chains. That will be also be … will be used in the future and these will detect the propensity of the light chain to form amyloid. We have to improve also the awareness, so the meeting that we are … we just have is very important, because awareness is critical to have the doctor think about the disease and use the appropriate tool, but first of all, he has to think about that, so awareness remain the really very important role.

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