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AL Amyloidosis

Authors:
  • Cynthia M. Johnson, MA
Publication Type:

Condition

AL Amyloidosis

(Amyloid Light Chain)

Definition

Amyloidosis is a buildup of abnormal proteins called amyloids. The body cannot break down these proteins. Instead, they build up in 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 (AL) light chain amyloidosis. It is an abnormal form of antibody proteins.

Causes

AL amyloidosis happens when cells in the bone marrow make abnormal antibodies. These antibodies misfold, bind together, and build up and cause damage to tissues and organs.

Risk Factors

AL amyloidosis 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
  • Monoclonal gammopathy of unknown significance

Symptoms

Problems from AL amyloidosis 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
Edema in Lower Legs.

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Diagnosis

The doctor will ask about symptoms and past health. 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:

Treatment

There is no cure for L amyloidosis. The goal of treatment is to manage symptoms and slow the disease. Options are:

  • Medicines that slow or stop new amyloids from forming, such as:
    • Chemotherapy medicines
    • Proteasome inhibitors
    • Immunomodulators
  • Supportive care, such as dietary changes
  • Radiation therapy
  • A stem cell transplant to replace current stem cells with healthy ones
  • 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.

Prevention

There are no current guidelines to prevent L amyloidosis.

RESOURCES:

CANADIAN RESOURCES:

CANADIAN RESOURCES:

References

  • AL Amyloidosis. Amyloidosis Foundation website. Available at: http://www.amyloidosis.org/facts/al.
  • Amyloid light chain (AL) amyloidosis. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/amyloid-light-chain-al-amyloidosis.
  • Amyloidosis. National Organization for Rare Disorders website. Available at: https://rarediseases.org/rare-diseases/amyloidosis.
  • 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.

Contributors

  • Daniel A. Ostrovsky, MD
Last Updated:
2023-06-01

(C) Copyright 2023 EBSCO Information Services

This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.