Know About "Amyloidosis"

Amyloidosis is a rare disease that occurs when a substance called amyloid builds up in your organs. Amyloid is an abnormal protein that is usually produced in your bone marrow and can be deposited in any tissue or organ.

Amyloidosis can affect different organs in different people, and there are different types of amyloid. Amyloidosis frequently affects the heart, kidneys, liver, spleen, nervous system and digestive tract. Severe amyloidosis can lead to life-threatening organ failure.

Types of Amyloidosis:
The type of protein and where it collects determines the type of amyloidosis you have. Amyloid deposits may collect throughout your body or in just one area.

There are different types of amyloidosis, including:

Primary (systemic AL) amyloidosis. This occurs without a known cause, but it has been seen in people with a blood cancer called multiple myeloma. This is the most common type of amyloidosis.

Secondary (systemic AA) amyloidosis. This is the result of another chronic inflammatory disease, such as lupus, rheumatoid arthritis, tuberculosis, inflammatory bowel disease (Crohn's disease and ulcerative colitis), and certain cancers. It most commonly affects the spleen, kidneys, liver, adrenal gland, and lymph nodes.

Dialysis-related amyloidosis (DRA). This is more common in older adults and people who have been on dialysis for more than 5 years. This form of amyloidosis is caused by deposits of beta-2 microglobulin that build up in the blood.

Familial, or hereditary, amyloidosis (AF). This is a rare form that is passed down through families. It is caused by an abnormal amyloid transthyretin (TTR) protein, which is made in the liver. This protein is responsible for the most common forms of hereditary amyloidosis.

Senile systemic amyloidosis (SSA). This is caused deposits of normal TTR in the heart and other tissues. It occurs most commonly in older men.

Organ-specific amyloidosis. This is cause deposits of amyloid protein in single organs, including the skin (cutaneous amyloidosis).

Symptoms of Amyloidosis:

    Changes in skin color
    Clay-colored stools
    Fatigue
    Feeling of fullness
    Joint pain
    Low red blood cell count (anemia)
    Shortness of breath
    Swelling of the tongue
    Tingling and numbness in legs and feet
    Weak hand grip
    Weakness
    Weight loss

Treatment:
There's no cure for amyloidosis. However doctor will prescribe treatments to suppress the development of the amyloid-forming protein, and to manage your symptoms.
High-dose chemotherapy with stem cell transplant can help remove the substance that leads to amyloid formation in those with primary AL amyloidosis who have no more than two major organs damaged.
Secondary (AA) amyloidosis is treated by controlling the underlying disorder and with powerful anti-inflammatory medicines called steroids, which fight inflammation.
Liver transplant may stop the disease in those with hereditary amyloidosis.
A kidney or heart transplant may also be recommended.