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AL

Amyloid light chain amyloidosis (AL)

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What is Amyloidosis?

Amyloidosis is a group of complex rare diseases caused by abnormal proteins that misfold and clump together to form amyloid deposits in tissues or organs, including the heart, kidney and peripheral nerves.1-3

This build up 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?

Amyloid light chain amyloidosis, also referred to as AL, is a systemic and progressive type of amyloidosis. In AL, a type of protein called light chains are produced abnormally by defective plasma cells in the bone marrow. These proteins misfold and form amyloid deposits.4,5

Accumulation of amyloid, particularly in the heart and kidneys, can cause progressive damage and may lead to premature death, most commonly due to cardiac failure or irregular heartrate (arrhythmia).4,5

Diagnosed prevalence in adults is:

Reference.6

~17K

In China

~14K

In Europe

~13K

In US

~2K

In Japan

AL often occurs from ages 50-80 but can be diagnosed in people as early as their late 20s.7 The median age of diagnosis is 60-67 years.7 About 60% of people diagnosed with AL are males.8

Symptoms:

People with AL may experience a range of signs and/or symptoms, including:9-11

Swollen arms and legs

Extreme shortness of breath

Abnormal heartbeat

Nausea

Diarrhoea

Tingling in the extremities

Carpal tunnel syndrome

Enlarged tongue

Rash around eyes

And many other vague symptoms

that mimic other diseases that often complicate diagnosis.

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References

  1. Mollee P, et al. How to diagnose amyloidosis. Internal Medicine Journal. 2014;44:7-17.
  2. Comenzo RL. How I treat amyloidosis. Blood. 2009;114(15):3147-3157.
  3. Witteles RM, et al. Screening for Transthyretin Amyloid Cardiomyopathy in Everyday Practice. JACC: Heart Failure. 2019;7(8):709-716.
  4. Desport E, et al. AL Amyloidosis. Orphanet J Rare Dis. 2012;7(54).
  5. Grogan M, et al. Light-chain cardiac amyloidosis: strategies to promote early diagnosis and cardiac response. Heart. 2017;103:1065-1072.
  6. 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 May 2024).
  7. Real de Asua D, et al. Systemic AA amyloidosis: epidemiology, diagnosis and management. Clinical Epidemiology. 2014; 6:369-377.
  8. Staron A, et al. Race/ethnicity in systemic AL amyloidosis: perspectives on disease and outcome disparities. Blood Cancer Journal. 2020:10:118.
  9. Wechalekar AD, et al. AL Amyloidosis for Cardiologists; Awareness, Diagnosis, and Future Prospects. JACC: CardioOncology. 2022;4(4): 427- 441.
  10. Amyloidosis - Symptoms and causes. (2020, March 14). Mayo Clinic. Accessed at: https://www.mayoclinic.org/diseases-conditions/ amyloidosis/symptoms-causes/syc-20353178. Accessed March 2021.
  11. Cuddy SAM, et al. Amyloidosis as a Systemic Disease in Context. Can J Cardiol. 2020:36:396-407.
  12. Dima D, et al. Diagnostic and Treatment Strategies for AL Amyloidosis in an Era of Therapeutic Innovation. JCO Oncology Practice. 2023;19(5):265-275.
  13. Dittrich T, et al. Prognosis and Staging of AL Amyloidosis. Acta Haematol. 2020;143:388-400
  14. Merlini G, et al. Systemic light chain amyloidosis: an update for treating physicians. Blood. 2013;121(26):5124-5130.
  15. 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/0012

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