Amyloidosis is a buildup of abnormal proteins called amyloids. The body cannot break down these proteins. Instead, they build up on tissues and organs like the heart, liver, and kidneys. This makes it hard for them to work as they should. This can be deadly. Early treatment can improve outcomes.
There are several types of amyloidosis based on the protein that is involved. This fact sheet focuses on amyloidosis (AL) light chain. It is an abnormal form of antibody proteins.
This problem happens when cells in the bone marrow make abnormal antibodies. These antibodies misfold, bind together, and build up on tissues and organs.
This health problem is most common in people who are 50 to 70 years of age.
Exposure to agent orange or certain weed killers may raise the risk of this health problem.
These health problems may also be linked to AL amyloidosis:
- Multiple myeloma
- Waldenstrom macroglobulinemia
Problems vary from person to person. Some common ones are:
- Weakness and lack of energy
- Weight loss
- Shortness of breath
- Enlarged tongue
Other symptoms will depend on the organs that are affected, such as:
- Kidneys: Swelling in the legs, belly, and arms
- Heart: Shortness of breath and abnormal heartbeat
- Digestive system: Nausea, diarrhea, lack of hunger, and feeling full
- Nerves: Lightheadedness; Tingling, pain, or numbness in the hands and feet
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The doctor will ask about your symptoms and health history. A physical exam will be done.
Blood and urine tests will be done to look for abnormal proteins and problems with organ function.
A sample of tissue or bone marrow may be taken to check for signs of the disease. This can be done with a biopsy .
Other testing may include:
There is no cure. The goal of treatment is to manage symptoms and slow the disease. Choices are:
Medicines that slow or stop new amyloids from forming, such as:
- Chemotherapy medicines
- Proteasome inhibitors
- Supportive care, such as dietary changes
- Radiation therapy
- A stem cell transplant to replace current stem cells with healthy ones from a donor
- Caring for specific organ problems, such as dialysis to ease stress on the kidneys
People who are not helped by these methods may need surgery. Deposits may be removed or an organ transplant may be needed.
Amyloidosis Foundation http://www.amyloidosis.org
Genetic and Rare Diseases Information Center http://rarediseases.info.nih.gov
Canadian Organization for Rare Disorders http://www.raredisorders.ca
The Kidney Foundation of Canada http://www.kidney.ca
AL Amyloidosis. Amyloidosis Foundation website. Available at: http://www.amyloidosis.org/facts/al. Accessed March 18, 2021.
Amyloid light chain (AL) amyloidosis. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/amyloid-light-chain-al-amyloidosis. Accessed March 18, 2021.
Amyloidosis. National Organization for Rare Disorders website. Available at: https://rarediseases.org/rare-diseases/amyloidosis. Accessed March 18, 2021.
Wechalekar AD, Gillmore JD, et al; British Committee for Standards in Haematology Committee. Guidelines on the management of AL amyloidosis. Br J Haematol. 2015 Jan;168(2):186-206.
What is amyloidosis? Boston University website. Available at: http://www.bu.edu/amyloid/about/what. Accessed March 18, 2021.
- Reviewer: EBSCO Medical Review Board Daniel A. Ostrovsky, MD
- Review Date: 12/2020
- Update Date: 00/31/2021