Amyloidosis

About Amyloidosis

Amyloidosis is a bone marrow disorder that produces an abnormal protein, called amyloid, that is then deposited in various organs and tissues of the body. Amyloidosis may be either primary (with no known cause), secondary (caused by another disease), or hereditary (passed down from parents to children). Localized amyloidosis may only affect a single tissue of the body, while systemic amyloidosis deposits may be found throughout the body, but most commonly affects the heart, liver, kidneys, spleen, nervous system or digestive tract; without treatment it can cause organ failure or death. The disease is typically diagnosed over the age of 40, and about two-thirds of patients are male.
Light chain amyloidosis (AL), is a disorder of the plasma cell, and is the most common form of Amyloidosis. Autoimmune amyloidosis (AA) occurs as a reaction to another chronic illness and is referred to as secondary or inflammatory amyloidosis. Hereditary or familial amyloidosis is rare, and is caused by inheriting a gene mutation; normally the harmful deposits don’t occur until adulthood. There are 2 classifications of hereditary amyloidosis which is considered a systemic disease, ATTR (transthyretin protein (TTR)), and Non-TTR. Leukocyte chemotactic factor 2 (LECT2), involves an amyloid subunit protein and is referred to as ALECT2. This disease is classified as an acquired systemic amyloidosis. At this time, the protein is thought to be part of cartilage reconstruction and tissue repair. Beta-2 microglobulin amyloidosis (AB2M) is associated with the B2M protein, and occurs with end stage renal (kidney) disease, resulting in the accumulation and deposit of this amyloid protein in bones, joints and tendons.