Amyloidosis is a condition involving the deposition of insoluble proteins within various tissues and organs of the body.

When certain protein monomers unravel into characteristic beta-pleated ribbon structures, partially denatured monomers are formed. These aberrantly folded proteins can act as seeds for recruiting additional monomers and triggering oligomerization. The resulting prefibrilar oligomers continue to elongate, eventually forming mature amyloid fibrils.

These amyloid fibrils are insoluble and can accumulate throughout the body, affecting one or more organs. As a result, the symptoms of amyloidosis depend upon which organs are involved and can be quite variable. In fact, the diagnosis of amyloidosis often starts with the detection of high protein levels in the urine. For proper diagnosis, however, an abdominal fat pad biopsy must be analyzed.

Amyloid proteins in the fat pad biopsy stained with Congo Red dye produce apple-green birefringence under polarized light, allowing for a confirmation of amyloidosis.

In the case of primary, or acute light-chain (AL) amyloidosis, the amyloid protein is derived from kappa or lambda immunoglobulin light chain molecules.

This can be positively identified by serum or urine protein electrophoresis to detect the presence of an M-spike. To further characterize the nature of the aberrant immunoglobulin, immunofixation is generally used to demonstrate whether the monoclonal gammopathy is derived from kappa or lambda light chains.

Transthyretin Amyloid Cardiomyopathy (ATTR-CM) is a rarely diagnosed fatal heart disease affecting an estimated 400,000 people globally, although the true prevalence is unknown and may be higher. Bayer is committed to optimize the care for patients living with this progressive disease, to prolong lives, and improve patients’ quality of life.

Pedro Vianna, MD, Dana-Farber Cancer Institute, Boston, MA, gives an overview of promising therapies under investigation for light chain (AL) amyloidosis, including anti-fibril monoclonal antibodies and bispecific antibodies. This interview took place at the 21st International Myeloma Society (IMS) Annual Meeting, held in Rio de Janeiro, Brazil.

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Mat Maurer, MD
Arnold and Arlene Goldstein Professor of Cardiology
Professor of Medicine
Columbia University Irving Medical Center, New York, NY

Presented at the 2023 Amyloidosis Support Groups ATTR/Hereditary Meeting, Saturday, October 28, 2023

In this video, Eyal Lebel, MD, Hadassah-Hebrew University Medical Center, Jerusalem, Israel, discusses the challenges in diagnosing light chain (AL) amyloidosis, emphasizing that it remains a rare and often underdiagnosed condition. Dr Lebel highlights the importance of raising awareness among various specialties, including family physicians, nephrologists, and cardiologists, as AL amyloidosis presents with multisystem involvement and symptoms that can easily be misattributed to other conditions. This interview took place at the 21st International Myeloma Society (IMS) Annual Meeting, held in Rio de Janeiro, Brazil.

These works are owned by Magdalen Medical Publishing (MMP) and are protected by copyright laws and treaties around the world. All rights are reserved.

In this video, Paolo Milani, MD, PhD, Amyloidosis Research and Treatment Center, Pavia, Italy, discusses the main predictors of early mortality in patients with wild-type transthyretin (ATTR) cardiac amyloidosis. Cardiac biomarkers such as cardiac troponin and NT-proBNP, along with patient age and performance status, help identify high-risk patients. This interview took place at the XIX International Symposium on Amyloidosis (ISA) in Rochester, MN.

These works are owned by Magdalen Medical Publishing (MMP) and are protected by copyright laws and treaties around the world. All rights are reserved.