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Amyloidosis Amyloidosis
by Kimberly Allen, RN Amyloidosis is a an umbrella term used to describe a group of diseases where an abnormal protein called amyloid is... Amyloidosis

by Kimberly Allen, RN

Amyloidosis is a an umbrella term used to describe a group of diseases where an abnormal protein called amyloid is produced and accumulates in your organs and tissues.  Amyloidosis is considered rare and frequently remains undiagnosed making it difficult to know just how many people are affected by this disease.  However, experts believe as many as 2,500 people develop the most common form of amyloidosis known as AL amyloidosis in the US every year.  Although amyloidosis can affect anyone at anytime it usually affects people over 60 years of age.  there are also certain types of amyloidosis that are inherited, these tend to manifest earlier.  Amyloidosis may also develop before 60 years of age in people with certain inflammatory conditions like rheumatoid arthritis or have a type of bone marrow cancer known as multiple myeloma.  there is also a form of amyloidosis that has been associated with dialysis, however, with the advances in dialysis techniques is much less common now.amyloidosis graph

There are a number of proteins that can cause the accumulation of amyloids, however, only a few are related to significant health problems.  Amyloid deposits can accumulate in just one or two  areas or throughout your entire body.  Doctors have divided amyloidosis into 3 major categories, primary or AL amyloidosis, secondary or AA amyloidosis and Familial or ATTR amyloidosis.

Primary or AL amyloidosis is the most common form of amyloidosis,  AL amyloidosis can affect numerous areas in your body including your organs like the kidneys and liver, even your heart and blood vessels.  The only disease known to be associated with this type of amyloidosis is multiple myeloma.  Otherwise the exact mechanism that causes this type is unknown.  Researchers may not know the exact cause yet, but they do know that  it starts in your bone marrow.  Most people think of bone marrow as producing red and white blood cells as well as platelets however, it also produces antibodies.  In a normal cycle your body breaks down the antibodies after they have served their purpose.  However, in AL ayloidosis the bone marrow produces antibodies that the body is unable to breakdown.  These antibodies then accumulate and eventually are deposited in your organs and tissues as amyloid.

Secondary or AA amyloidosis is caused by the A protein and develops due to another chronic inflammatory or infectious disease like tuberculosis or rheumatoid arthritis, even certain type of cancer can cause AA amyloidosis.  This type of amyloidosis usually affects the spleen, liver, and kidney’s as well as the adrenal glands and lymph nodes.

Familial or ATTR amyloidosis is the rarest form of amyloidosis and is passed on through families.  It is caused by the protein amyloid transthyretin thus the name ATTR.

The symptoms of amyloidosis depends on which organs and tissues are involved and may include swelling in your legs and ankles, severe fatigue and weakness, an enlarged tongue, difficulty swallowing and weight loss.  you may also experience changes in your skin color as well as the color of your stools.  many also experience an irregular heartbeat and shortness of breath as well as numbness and tingling in their hands and feet.

There is no cure currently available for amyloidosis.  The goal of treatment is to manage the symptoms and curb the production of amyloid proteins.  For people with primary amyloidosis the doctor may recommend certain chemotherapy medications including Cytoxan and Alkeran.  There are also other types of chemotherapy that are being tested to determine if they are effective in treating amyloidosis.  Ask your doctor about any clinical trials that are currently available.  Another treatment being used is peripheral blood stem cell transplantation.  This procedure is not for everyone, it has serious risks including death and should be thoroughly researched and discussed with your doctor before considering it.  In people with secondary amyloidosis the goal of treatment is to treat the underlying condition that has caused your amyloidosis.  For people with hereditary amyloidosis the protein that causes this type of amyloidosis is produced in the liver so many doctors feel a liver transplant may be the treatment of choice.  Researchers are also investigating new medications that can stop the progression of this type of amyloidosis.

Kimberly Allen is a registered nurse with an AND in nursing. She has worked in ACF, LCF and psychiatric facilities, although she spent most of her career as a home health expert. She is now a regular contributor to HealthAndFitnessTalk.com, dispensing advice and knowledge about medical issues and questions. You can reach her with any comments or questions at  mussatti3@gmail.com.

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