Recent developments in systemic light-chain amyloidosis prognosis and treatment
1National Amyloidosis Centre, University College London, London, UK.
Future Cardiology
|July 12, 2025
Summary
Systemic light-chain amyloidosis (AL) is a rare plasma cell disorder. New therapies target amyloid deposits, offering hope for improved outcomes, especially in patients with cardiac involvement.
Area of Science:
- Hematology
- Cardiology
- Oncology
Background:
- Systemic light-chain (AL) amyloidosis is a rare plasma cell disorder.
- Abnormal immunoglobulin free light-chains misfold into amyloid fibrils, depositing in tissues.
- Cardiac involvement is a primary determinant of prognosis in AL amyloidosis.
Purpose of the Study:
- To review advancements in diagnostics and treatment for AL amyloidosis.
- To highlight the role of multimodality cardiac imaging.
- To discuss novel anti-amyloid therapies targeting fibril removal.
Main Methods:
- Review of current literature on AL amyloidosis pathophysiology, diagnostics, and therapeutics.
- Emphasis on advancements in cardiac imaging techniques.
- Discussion of emerging anti-amyloid therapies.
Main Results:
- Multimodality cardiac imaging improves diagnosis and treatment monitoring.
- Targeting the plasma cell clone has transformed treatment.
- Novel therapies aimed at amyloid fibril clearance are in development.
Conclusions:
- Systemic AL amyloidosis treatment has significantly advanced.
- Cardiac imaging plays a crucial role in managing AL amyloidosis.
- Emerging therapies hold promise for disease regression by clearing amyloid deposits.
Related Concept Videos
01:26Nephrotic Syndrome II : Assessment and Medical Management
23
IntroductionNephrotic syndrome is a kidney disorder marked by excessive protein loss in the urine, leading to various systemic complications. This condition often results from damage to the glomeruli—the kidney's filtering units—causing proteinuria, low blood protein levels, and fluid retention. Understanding the assessment, diagnosis, and management of nephrotic syndrome is essential for effective treatment and prevention of further kidney damage.AssessmentPatient History: Document...
23
01:20Myasthenia Gravis: Overview and Treatment
2.0K
Myasthenia gravis is a neuromuscular transmission disorder characterized by weakness and increased fatigability of skeletal muscles. It is an autoimmune disease affecting approximately one in 2000 people, where antibodies against the α1 subunit of nicotinic acetylcholine receptors are produced.
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which...
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which...
2.0K
02:57Targeted Cancer Therapies
7.8K
The targeted cancer therapies, also known as “molecular targeted therapies,” take advantage of the molecular and genetic differences between the cancer cells and the normal cells. It needs a thorough understanding of the cancer cells to develop drugs that can target specific molecular aspects that drive the growth, progression, and spread of cancer cells without affecting the growth and survival of other normal cells in the body.
There are several types of targeted therapies against...
There are several types of targeted therapies against...
7.8K
01:22Alzheimer's Disease: Treatment
263
Alzheimer's Disease (AD), a neurodegenerative disorder, is pathologically identified by amyloid plaques and neurofibrillary tangles composed of tau protein. AD pharmacotherapy aims to manage cognitive symptoms, delay disease progression, and treat behavioral symptoms. The treatment is primarily symptomatic and palliative, with no definitive disease-modifying therapy available. Cholinesterase inhibitors, including donepezil (Aricept), rivastigmine (Exelon), and galantamine (Razadyne), are...
263
03:03Amyloid Fibrils
9.9K
Amyloid fibrils are aggregates of misfolded proteins. Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils.
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining,...
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining,...
9.9K
01:26Atherosclerosis III: Management
40
Management of atherosclerosis involves an integrated strategy encompassing pharmacological treatment, surgical interventions, lifestyle changes, and nutrition therapy to address the multifactorial nature of the disease.Pharmacological TherapyA cornerstone of atherosclerosis management is the use of pharmacological agents. Statins, such as atorvastatin, are pivotal in inhibiting HMG-CoA reductase, an enzyme that catalyzes an initial step in cholesterol synthesis in the liver. This reduction in...
40