by Kimberly Allen RN
Amyloidosis is a term used for a group of disorders in which abnormal proteins called amyloids are mistakenly deposited into the tissues and/or organs. These abnormal proteins or amyloids are usually made by cells in the bone marrow and can be deposited in any organ or any tissue throughout the body. Amyloidosis is considered rare with only 1,500 to 3,500 newly diagnosed cases every year.
Amyloidosis can affect anyone at anytime, however it is more commonly diagnosed in people over 60 years of age. People with certain disease like chronic inflammatory or infectious diseases as well as people with certain cancers like multiple myeloma also have an increased chance of developing amyloidosis. Amyloiosis has also been associated with dialysis. Dialysis is unable to remove the large amyloid proteins therefore they build up in the surrounding tissue. However, with improved dialysis techniques the incidence of amyloidosis has decreased.
In addition to the dialysis related amyloidosis there are 3 main types of amyloidosis;
1. Primary Amyloidosis (AL) develops when a particular cell called a plasma cell in the bone marrow starts overproducing certain proteins that are part of an antibody called the light chain. With the exception of multiple myeloma this type of amyloidosis is not associated with other diseases but is considered an independent disease entity.
2. Secondary Amyloidosis however develops as a result or complication of another disease including chronic inflammatory diseases like RA or chronic infections like TB. This type can also develop secondary to multiple myeloma.
3. Familial Amyloidosis known as ATTR is a form of inherited amyloidosis that is quite rare. This type of amyloidosis is an autosomal dominant inherited condition which means that all children of a person with this type of amyloidosis have a 50% chance of also inheriting the disease.
There are also several types of localized amyloidosis. In these types of amyloidosis the amyloid protein deposits are found in specific area of the body and are different than the amyloid proteins that are deposited throughout the body.
The symptoms of amyloidosis vary depending on the organ or organs affected. Because it can affect many different organs including the heart, lungs , liver, kidneys, etc the symptoms may also be vague and not noticeable in the early stages. However, when symptoms do manifest they usually include weakness and fatigue, shortness of breath, poor appetite and weight loss.
As there is no cure for amyloidosis researchers have been studying several different medications including the use of Alkeran or Cytoxin as well as dexamethasone as these medications disrupt the growth of abnormal cells that produce amyloid proteins. Researchers are also studying several other chemotherapy medications to determine if they are effective in treating and managing amyloidosis. Ask your Dr if any of these medications might be appropriate for you or if there are any clinical trials that may be available.
Another treatment that is currently being uses with good results is peripheral blood stem cell transplant. These are considered aggressive therapies and are not for everyone. Stem cell transplants carry significant risks including death so you should discuss your options thoroughly with your Dr and weigh the benifits and risks before making any decisions.