Primary Types of Amyloidosis

Primary Types of Amyloidosis

Amyloidosis is a disease in which abnormal proteins (amyloids) build-up in the body’s tissues and organs. These once soluble proteins develop fragments that combine to form insoluble fragments or fibers. These insoluble fibers are then deposited in different organs and tissues in the body causing damage to those organs and tissues causing many other diseases. In this article we will talk about three different types of amyloidosis, what they do, what to look for and ways to treat them:

1. Light chain amyloidosis (AL)

 Light chain amyloidosis also known as AL amyloidosis, formerly known as primary amyloidosis is the most common type of amyloidosis in the United States. The reason it is referred to as light chain amyloidosis is because it starts in the bone marrow which produces plasma cells. The plasma cells have antibodies called immunoglobulin which are the proteins that make up the antibodies. These immunoglobulins are made up of two light protein chains and two heavy protein chains. The plasma in a person with amyloidosis will form irregular protein chains, specifically the light ones, and those irregular chains will bind together to form an amyloid. From there the amyloids are placed throughout the body and build-up where they are deposited causing damage to those areas. It can affect many organs and tissues but it is not known to cause any effects on the brain.

2. Autoimmune amyloidosis 

Autoimmune amyloidosis also known as secondary amyloidosis and inflammatory amyloidosis is when the amyloid proteins build up in the tissues. It happens in conjunction with other chronic diseases such as diabetes (excess glucose in the blood and urine), tuberculosis(contagious, infectious growths on the lungs), rheumatoid arthritis (deformity, immobility and pain of the extremities), and inflammatory bowel disease (inflammation the bowels).

3. Hereditary amyloidosis

Hereditary amyloidosis is a type of systemic disease caused by inheriting a gene mutation. The mutation produces abnormal amyloid proteins that go out to the nervous system and other organs in the body and build-up. This for of amyloidosis has two main classifications which are ATTR and Non-TTR. ATTR amyloidosis is transthyretin amyloid. It causes the protein transthyretin to be unstable. In doing so it causes the amyloid protein to fold incorrectly. These deformed proteins then go out to the body’s nervous system and organs. The main form of treatment for amyloidosis is to treat the symptoms and damage and to slow down or stop the excessive production of amyloid proteins at the source of the disease. For certain cases liver transplants are performed because the liver in ATTR is the main source of amyloid production. The most common symptoms are weight loss, fatigue, and swelling of the spleen, liver, thyroid, and other various areas in the body. Some of the other areas that can be affected are the kidneys, heart, the digestive system, and nervous system. Testing for amyloidosis requires that a person is tested for amyloid proteins in the body. This is done with blood tests, urine tests, and biopsies to determine and treat the disease.

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