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What are the Clinical Features of Systemic Amyloidosis?

Delayed diagnosis of amyloidosis, which pushes back the start of treatment, increases the mortality risk and negative quality of life for patients. It is one of the biggest issues for this disease today. Dr. Joselle Cook, hematologist from the Mayo Clinic, and Dr. Hira Shaikh, hematologist from the University of Iowa Healthcare, discuss the findings from a systematic scoping review they led during the two-year development of the American Society of Hematology AL Amyloidosis Guidelines. It was identified relatively early on that a gap exists as to WHEN clinicians could begin to suspect systemic amyloidosis and WHAT were early signs and symptoms that clinicians may see that could prompt testing for the disease. This is a must-watch video for all frontline clinicians, regardless of specialty.

 

To read the paper associated with this video:

“Clinical Features of Systemic Amyloidosis: A Scoping Review”

To read the paper associated with the same ASH (American Society of Hematology) 2-year project:

“2026 Guidelines on Diagnosis of Light Chain Amyloidosis”

 

Navigating Mental Health Challenges in Cardiac Amyloidosis

Dr. David Rintell is a licensed psychologist with experience working with individuals and families living with long-term illness. In this video Dr. Rintell, in his personal expert capacity,  explores what we know about mental health, depression, and anxiety in those with cardiac amyloidosis. He offers two items to screen for depression, how it may impact caregivers as well, and treatment considerations. In closing, he discusses how quality of life is often impacted and ways to address it.

Kidney Transplantation for Patients with AL Amyloidosis

Dr. Heather Landau, Director of the Amyloidosis Program at Memorial Sloan Kettering, gives an excellent lecture on why now is the time to consider kidney transplantation for AL amyloidosis patients. She details why the determination is complex and weighty, while offering the offsetting balance of daily and long-term implications of renal replacement therapy. Dr. Landau goes on to discuss “Why now?”, pre-transplant evaluation, and which AL amyloidosis patients would lead the kidney transplant consideration. She concludes the lecture with post-transplant management/follow-up recommendations.

Clinical Utility of AI Based Review of Echocardiography for Diagnosis of Cardiac Amyloidosis

Dr. Faizi A. Jamal, Chief, Division of Cardiology and Director, Non-Invasive Cardiology Laboratory at the City of Hope National Medical Center has been at the forefront of evaluating the clinical utility of AI based review of echocardiography in the diagnosis of cardiac amyloidosis. In this video he shares his experience with Ultromics’s EchoGo Amyloidosis, and how the City of Hope has incorporated this into their clinical workflow. He discusses the typical initial reasoning for ordering echocardiograms, which is to understand the severity of issues such as HFpEF or Aortic Stenosis, and how AI can take the analysis further to explore whether cardiac amyloidosis may be an underlying cause of the condition. Dr. Jamal discusses the multiple clinical challenges to diagnosing cardiac amyloidosis and details the numerous benefits experienced from the AI based review of echocardiograms. “EchoGo Amyloidosis is going to be revolutionary, based upon the volume of undiagnosed patients that are out there. It’s going to undoubtedly detect patients and impact morbidity and mortality for this disease.”

Click HERE, or on graphic below to view Dr. Jamal’s video.

Ejection Fraction: It’s Just a Fraction

Dr. Julie Rosenthal, Director of the Cardiac Amyloidosis Program at Mayo Clinic in Arizona, introduces us to the cardiovascular system and summarizes cardiac amyloidosis. She then discusses the echocardiogram and how it is used to look for abnormalities, such as thickening of the heart wall and pericardial effusion. Animated patient videos clearly convey how an impaired amyloid heart looks from multiple perspectives. She offers a tutorial on ejection fraction, what it is, how it is calculated, and why not all ejection fractions are the same despite the numbers. Importantly, she highlights that stroke volume is actually the more meaningful measurement of a patient’s cardiac output and why.

Weeds in the Garden: ABCs of AL Amyloidosis

Dr. Morie Gertz, hematologist, professor of medicine at the Mayo Clinic in Rochester and world renowned expert in amyloidosis, provides the ABCs of AL amyloidosis. His “Weeds in the Garden” is a legendary story explaining the biology behind this disease and how mis-folded proteins are created and evolve. He concludes with how treatments seek to deal with these mis-folded proteins and the goal of improving patients’ quality of life. This is a must-watch video for healthcare professionals from an esteemed expert.

Do You Need a Heart Biopsy to Diagnose Cardiac Amyloidosis?

Dr. Ahmad Masri, Director of the Cardiac Amyloidosis Program at Oregon Health & Science University (OHSU), reminds us about the traditional approach to diagnosis of cardiac amyloidosis. Unfortunately, this has not been enough. Thankfully, over the past decade that has all changed. He talks about OSHU’s approach to diagnosis today and what other data is used to arrive at a diagnosis of cardiac amyloidosis, offering four sample patient cases for insights.

Autologous Stem Cell Transplantation in AL Amyloidosis: what, why, when, and for whom?

Dr. Heather Landau is an oncologist and hematologist at the Memorial Sloan Kettering Cancer Center, bone marrow transplant specialist, and director of their Amyloid Program. In this video Dr. Landau reviews the history and goal of treatment for AL amyloidosis. Next she delves into detail and experiences of using high dose therapy and autologous stem cell transplant to treat light chain (AL) amyloidosis. In conclusion, she will discuss how the treatment landscape has changed based on recent advances.

Harnessing Your Immune System: CAR-T and Bispecific Antibodies

Dr. Morie Gertz, professor of medicine at the Mayo Clinic in Rochester and world renowned expert in amyloidosis, talks about how the immune system can be harnessed in the fight against amyloidosis and multiple myeloma. He discusses two immune-directed therapies: CAR-T and Bispecific Antibodies. Dr. Gertz eloquently, and in an easy-to-understand way, summarizes the goal of immune-directed therapies and the two approaches today, including the processes, outcomes, advantages, and risks to be considered. This is a must-watch video for physicians from a legendary expert.

ATTR Amyloidosis Treatments: Stabilizers and Silencers

Dr. Brett Sperry, cardiologist and director of the Cardiac Amyloidosis Program at Saint Luke’s Mid America Heart Institute, provides an excellent overview of FDA-approved ATTR amyloidosis treatments. He goes into detail about the biology behind silencers and stabilizers and exactly how they impair amyloidosis progression. In addition, he previews the future, summarizing new categories of drugs on the horizon.

Update: In November, 2024 the FDA approved Attruby (Acoramidis) for ATTR-CM (wild-type and hereditary/variant).

The future is indeed exciting!

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