Amyloidosis

The OHSU Knight Cancer Institute is at the forefront of treating and studying amyloidosis, a rare blood disorder that is similar to cancer. We offer you:

  • A team-based approach, with an array of specialists who combine their expertise.
  • Oregon’s largest bone marrow transplant center, with the state’s only donor-based transplants.
  • A nurse coordinator who can arrange your appointments and answer your questions.
  • A full range of support services, including an amyloidosis support group where you can meet with doctors and other patients and families.
  • Access to treatments not yet widely available, through clinical trials offered in cooperation with national research groups.

Understanding amyloidosis

What is amyloidosis?

Amyloidosis is a group of serious disorders that are not cancer but that can be linked to some types of cancer. These disorders occur when your bone marrow produces abnormal proteins called amyloid fibrils. These proteins build up in tissues and organs, eventually causing damage and impairing their function. Researchers don’t know why the body makes these proteins.

Who gets amyloidosis?

Amyloidosis is rare. An estimated 4,500 people in the U.S. are diagnosed with the most common type, AL (or light chain) amyloidosis, each year. Overall, fewer than 200,000 people in the U.S. are living with it. Risk factors include:

  • Gender: Men are at higher risk.
  • Age: Most patients are 40 or older at diagnosis.
  • Family history: One type of amyloidosis is linked to hereditary genetic changes.
  • Other conditions: Having multiple myeloma or another type of blood cancer raises risk.
  • Treatment: Having long-term kidney dialysis increases risk.

Outcomes depend on the specific type of amyloidosis and the organs and tissues affected. With effective treatment, many patients live with amyloidosis for decades. 

Amyloidosis symptoms

Amyloidosis may not cause noticeable symptoms until it is advanced enough to affect your organs. Symptoms vary depending on the organ affected. They may include:

  • Appetite changes, weight loss, diarrhea or constipation
  • Fatigue, weakness and shortness of breath
  • Irregular heartbeat
  • Joint pain, numbness or tingling in the legs, feet, hands or wrists
  • Skin changes such as bruising

Diagnosing amyloidosis

A physical exam and medical history are the first steps . Your care team may also recommend:

Blood and urine tests: These tests can verify a diagnosis and determine which organs are affected. A test called free light chain assay specifically looks for abnormal amyloid proteins in the blood.

Imaging tests: Scans can detect amyloid fibrils in the heart and other organs, and check for damage. Imaging tests may include:

  • Echocardiogram, a test that uses sound waves to show heart motion and blood flow.
  • MRI (magnetic resonance imaging), which uses magnetic fields and radio waves to create detailed images of structures inside the body.

Biopsy: Doctors may use a thin needle to collect a tissue sample so they can look for amyloid deposits. They may also look at bone marrow tissue or fluid to check for abnormal protein-producing plasma cells.

Genetic testing: If doctors suspect you have an inherited form of amyloidosis, they may refer you to our genetic testing and risk assessment experts, who can help you understand your condition and guide you through next steps.

Types of amyloidosis

Amyloidosis types include:

AL (amyloid light chain) amyloidosis: This is the most common type. It’s also known as primary amyloidosis. Light chains are proteins that become misfolded, forming the amyloid fibril. About 15% of patients with multiple myeloma, a blood cancer, also have AL amyloidosis.

AL amyloidosis may affect the:

  • Digestive system
  • Heart
  • Kidneys
  • Liver
  • Nerves
  • Skin

AA (amyloid A protein) amyloidosis: This type, also known as secondary amyloidosis, can develop as the result of an inflammatory disease, such as rheumatoid arthritis, or chronic infection. It often affects kidneys and sometimes the heart and liver.

Hereditary amyloidosis: This is an inherited disorder also known as ATTR or familial amyloidosis. It occurs when a gene mutation causes misfolding of the amyloid protein. ATTR stands for the amyloid transthyretin (TTR) protein. 

Hereditary amyloidosis tends to affect the:

  • Heart
  • Kidney
  • Liver
  • Nerves

Dialysis-related amyloidosis: This type affects people who have spent a long time on dialysis, a procedure to clear toxins from people with poor kidney function. Amyloid proteins are deposited in joints and tendons, causing symptoms such as pain and numbness in fingers.

Localized amyloidosis: In this type, abnormal proteins collect in just one area, such as the airway, eye or bladder.

Amyloidosis treatments

Knight Cancer Institute doctors lead national groups that focus on improving our understanding and treatment of amyloidosis. Our specialists also work together to make sure we consider every option available for your care.

Depending on your needs, your team might include experts such as:

  • Hematologist-oncologists (doctors who are experts in blood and cancer, and who treat cancer with chemotherapy)
  • Cardiologists (heart doctors)
  • Nephrologists (kidney doctor)
  • Gastroenterologists (digestive system doctors)
  • Neurologists (brain, spinal cord and nerve doctors)

Treatments vary depending on the type and severity of your amyloidosis, and on the organs involved. We may recommend:

Chemotherapy: Single or combined medications are given by mouth or injected into a muscle, into a vein or under the skin. They circulate in the bloodstream to kill abnormal cells. To improve effectiveness, chemotherapy drugs may be used in combination with other medications, such as targeted therapies.

Targeted therapy: For amyloidosis, medications called proteasome inhibitors take aim at proteasomes. Proteasomes are groups of proteins in all cells. Blocking them in problem cells allows abnormal proteins to build up, eventually destroying the cell.

Radiation therapy: To destroy amyloid deposits limited to a specific area, such as the airway or bladder, we may use external beam radiation therapy. A machine delivers highly targeted beams of energy to attack abnormal cells. OHSU was the first center in Oregon to use sophisticated, image-guided radiation therapy to precisely target abnormal cells while avoiding healthy tissue.

Bone marrow transplant: OHSU’s state-of-the-art bone marrow transplant center  is the largest in Oregon, giving us unmatched expertise in these complex procedures. Your doctors first destroy diseased plasma cells with chemotherapy or radiation therapy. Next, a specialist transplants healthy new cells to help fight infection and rebuild your immune system.

Interior photo of the Knight Cancer Research Building
The Knight Cancer Research Building, completed in 2018, includes state-of-the-art labs and collaborative spaces.

Amyloidosis clinical trials and research

As the only academic medical center in Oregon, OHSU is committed to applying the latest research developments to our patients’ care.

Clinical trials: We offer access to leading-edge clinical trials for amyloidosis at every stage. We also coordinate with other institutions to study amyloidosis and to test promising new therapies.

Immunotherapy research: One avenue of research looks at medications called monoclonal antibodies. These medications are designed to target specific markers on the cells that make some types of amyloidosis proteins.

Learn more

For patients

Call 503-494-7999 to:

  • Request an appointment
  • Seek a second opinion
  • Ask questions

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Center for Health & Healing Building 2
3485 S.W. Bond Ave.
Portland, OR 97239
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