What is Amyloidosis?
Amyloidosis is a group of complex rare diseases caused by abnormal proteins that misfold and clump together to form amyloid fibrils that are deposited in tissues or organs, including the heart, kidney and peripheral nerves.1-3
This deposit buildup can result in significant organ damage and organ failure that can severely impact quality of life and can ultimately be fatal.1-3
What is AL Amyloidosis?
Light chain amyloidosis, also referred to as AL amyloidosis, is a systemic and progressive type of amyloidosis. In AL amyloidosis, a type of protein called light chains are produced abnormally by defective plasma cells in the bone marrow. These proteins misfold and form amyloid fibril deposits.4,5
Accumulation of deposited amyloid fibrils, particularly in the heart and kidneys, can cause progressive damage and may lead to premature death, most commonly due to cardiac failure or irregular heart beat (arrhythmia).4,5
There are two subtypes of AL amyloidosis: kappa light chain amyloidosis (~20% of patients) and lambda light chain amyloidosis (~80% of patients).6-8
Diagnosed prevalence in adults is:
Reference.9
In US
In Japan
The median age of diagnosis is 64 years, but people can be diagnosed with AL amyloidosis
as early as their late 20s.10,11 About 60% of people diagnosed with AL amyloidosis are
males.12
Symptoms:
People with AL amyloidosis may experience a range of signs and/or symptoms, including: 13,15, 16
Swollen arms and legs
Extreme shortness of breath
Abnormal heartbeat
Nausea
Diarrhoea
Tingling in the extremities
Carpal tunnel syndrome
Enlarged tongue
Bruising around the eyes
And many other vague symptoms
that mimic other diseases that often complicate diagnosis.
References
- Mollee P, et al. How to diagnose amyloidosis. Internal Medicine Journal. 2014;44:7-17.
- Comenzo RL. How I treat amyloidosis. Blood. 2009;114(15):3147-3157.
- Witteles RM, et al. Screening for Transthyretin Amyloid Cardiomyopathy in Everyday Practice. JACC: Heart Failure. 2019;7(8):709-716.
- Desport E, et al. AL Amyloidosis. Orphanet J Rare Dis. 2012;7(54).
- Grogan M, et al. Light-chain cardiac amyloidosis: strategies to promote early diagnosis and cardiac response. Heart. 2017;103:1065-1072.
- Kastritis E, et al. ANDROMEDA Trial Investigators. Daratumumab-based treatment for immunoglobulin light-chain amyloidosis. N Engl J Med. 2021;385(1):46-58. DOI: 10.1056/NEJMoa2028631.
- Manwani R, et al. A prospective observational study of 915 patients with systemic AL amyloidosis treated with upfront bortezomib. Blood. 2019;134(25):2271-2280.DOI: 10.1182/blood.2019000834.
- Staron A, et al. Marked progress in AL amyloidosis survival: a 40-year longitudinal natural history study. Blood Cancer J. 2021;11(8):139. DOI: 10.1038/s41408-021-00529-w.
- AstraZeneca Data on File - Epidemiology estimates are composed of a triangulation of different data sources including Data Monitor, Decision Resources Group, Kantar Health, and internal input (updated as of July 2026).
- Baker KR. Light chain amyloidosis: epidemiology, staging, and prognostication. Methodist Debakey Cardiovasc J. 2022;18(2):27-35.
- Singh A, et al. Timing and co-occurrence of symptoms prior to a diagnosis of light chain (AL) amyloidosis. Blood Cancer J. 2024;14(1):61.
- Staron A, et al. Race/ethnicity in systemic AL amyloidosis: perspectives on disease and outcome disparities. Blood Cancer Journal. 2020:10:118.
- Wechalekar AD, et al. AL Amyloidosis for Cardiologists; Awareness, Diagnosis, and Future Prospects. JACC: CardioOncology. 2022;4(4): 427- 441.
- Anand S, et al. Nephrol Dial Transplant. 2024;40[1]:34-47.
- Amyloidosis - Symptoms and causes. (2020, March 14). Mayo Clinic. Accessed at: https://www.mayoclinic.org/diseases-conditions/ amyloidosis/symptoms-causes/syc-20353178. Accessed August 2026.
- Cuddy SAM, et al. Amyloidosis as a Systemic Disease in Context. Can J Cardiol. 2020:36:396-407.
- Lyons G, et al. Open Heart. 2025;12[1]:e003124.
- McCausland KL, et al. Patient. 2018;11[2]:207-216.
- Dittrich T, et al. Prognosis and Staging of AL Amyloidosis. Acta Haematol. 2020;143:388-400.
- Merlini G, et al. Systemic light chain amyloidosis: an update for treating physicians. Blood. 2013;121(26):5124-5130.
- Palladini G, et al. A staging system for renal outcome and early markers of renal response to chemotherapy in AL amyloidosis. Blood. 2014;124(15):2325-2332.
Veeva ID: GL/UNB-AML/0030