Amyloidosis: Introduction

Approved by the Cancer.Net Editorial Board, 09/2021

ON THIS PAGE: You will find some basic information about an illness called amyloidosis, which is not cancer, and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to Amyloidosis. Use the menu to see other pages. Think of that menu as a roadmap to this entire guide.

About amyloidosis

Amyloidosis is a rare and serious illness. Amyloidosis occurs when normal proteins in the body become misshapen and clump together. These misshapen proteins are called amyloid deposits or fibrils. Over time, the amyloid deposits build up in organs and tissues in the body. Eventually, this buildup cause symptoms and organ failure because the organs and tissues are not able to work as well as they should.

Amyloid protein deposits can be found in specific organs, such as the lung, skin, bladder, or bowel, or they can be systemic. "Systemic" means that the deposits may be found throughout the body. Systemic amyloidosis is the most common.

Amyloidosis is a rare disorder. Although it is not a type of cancer, it may be associated with certain blood cancers like multiple myeloma. Because amyloidosis is rare, it has been difficult to study. However, doctors and researchers have begun to understand more about amyloidosis over the past few decades. Research continues to learn more about this illness, which can be life-threatening.

Types of amyloidosis

There are different types of amyloidosis. They are named for the type of protein that is affected by the illness. The types of amyloidosis include:

  • AL amyloidosis. This is the most common type of amyloidosis in the United States. AL amyloidosis is a disorder of the plasma cells. Plasma cells are a type of white blood cell responsible for the production of immunoglobulins or antibodies, which are a type of protein that fights infection. The amyloid proteins that build up in the tissues in this condition are known as light chains. They can either be kappa or lambda light chains. "AL" stands for amyloid and light chain.

    In AL amyloidosis, the light chain proteins are misshapen and there are too many of them. These are deposited in tissues and can damage one or more organs. The heart, kidneys, nerves, and gastrointestinal system are the most common organs affected. Because AL amyloidosis is associated with the overproduction of plasma cell proteins, it is linked to multiple myeloma.

  • AA amyloidosis. AA amyloidosis is also called "autoimmune amyloidosis," "secondary amyloidosis," or “inflammatory amyloidosis.” In this condition, the amyloid protein that builds up in the tissues is called the A protein. AA amyloidosis is associated with some chronic diseases, such as diabetes, tuberculosis, rheumatoid arthritis, and inflammatory bowel disease. It may also be linked to aging. AA amyloidosis can affect the spleen, liver, kidneys, adrenal glands, and lymph nodes. Lymph nodes are tiny, bean-shaped organs that fight infection.

  • Hereditary or familial amyloidosis. Hereditary amyloidosis is rare. It can be passed from generation to generation within a family. The proteins produced in hereditary amyloidosis may cause problems with the heart and may cause carpal tunnel syndrome and eye abnormalities. The most common subtypes involve a protein called transthyretin (TTR). This is sometimes called ATTR amyloidosis.

This section covers AL, AA, and hereditary amyloidosis. Other types of amyloidosis include beta-2 microglobulin amyloidosis, which occurs in some patients with chronic kidney problems, and types of amyloidosis located in specific areas of the body.

The next section in this guide is Statistics. It helps explain how many people are diagnosed with amyloidosis and general survival rates. Use the menu to choose a different section to read in this guide.