Definition

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.

Causes

This problem happens when cells in the bone marrow make abnormal antibodies. These antibodies misfold, bind together, and build up on tissues and organs.

Risk Factors

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

Symptoms

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

Edema in Lower Legs

Copyright © Nucleus Medical Media, Inc.

Diagnosis

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:

• Serum amyloid P component (SAP) scintigraphy to take images to look for amyloid deposits
• Electrocardiography or echocardiogram to check heart function

Treatment

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
  • Immunomodulators
• 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.

Prevention

There are no current guidelines to prevent this health problem.