Systemic amyloidosis is a serious condition caused by the accumulation of amyloid deposits in soft tissues, impairing organ function and leading to organ failure. Treatment involves drugs to inhibit antibody production and relieve inflammation, and a bone marrow transplant. Diagnosis is confirmed with a tissue biopsy, and symptoms include fatigue, discomfort, and organ dysfunction. Treatment focuses on managing symptoms, and stem cell transplantation may be performed. There is no known cause or cure for systemic amyloidosis.
Systemic amyloidosis is a serious condition defined by the widespread accumulation of amyloid deposits throughout the body. Associated with the presence of abnormal antibodies produced in the bone marrow, systemic amyloidosis can impair organ function contributing to organ failure. Treatment generally involves the use of drugs, designed to inhibit the production of antibodies and relieve inflammation, and a bone marrow transplant. There is no cure for systemic amyloidosis.
A diagnosis of systemic amyloidosis is usually confirmed with a tissue biopsy. Often obtained under local anesthesia, from the skin or bone marrow, a biopsy usually shows elevated amyloid proteins. As a precaution, urine and blood tests may also be done to rule out the presence of other conditions that may mimic amyloidosis.
Originating in the bone marrow, there is no known cause for developing amyloidosis. The bone marrow naturally produces versatile antibodies, designed to fight foreign pathogens, which are re-assimilated once the threat is eliminated. In the case of amyloidosis, the antibodies produced are mutated and cannot be metabolized or broken down. With nowhere else to go, the antibodies travel throughout the body via the bloodstream and turn into amyloid protein before settling in soft tissue.
The widespread accumulation of amyloids can interfere with system and organ functions, especially when the nervous, respiratory, and digestive systems are affected. Because systemic amyloidosis affects several soft tissues simultaneously, individuals experience a variety of signs and symptoms. Individuals may tire easily and experience discomfort, including numbness and swelling in the extremities. When the digestive system is affected, individuals develop a decreased appetite, irregular bowel movements, and unintentional weight loss. Additional signs may include arrhythmia, decreased kidney function, and difficulty breathing.
In the absence of a cure, treatment for systemic amyloidosis focuses on managing symptoms. A combination of medications, including steroid medications such as dexamethasone, are usually given to relieve inflammation and discomfort. If the accumulation of amyloid significantly impairs organ function, organ failure can occur. Complications, such as respiratory and renal failure, are not uncommon and may require additional and extensive treatment to slow the progression of symptoms.
In some cases, blood stem cell transplants, usually harvested from your own stem cells, and bone marrow transplants may also be performed. Transplantation allows for a replenishment of healthy antibodies to replace mutated and diseased ones and inhibit the production of new abnormal amyloids. Stem cell transplantation carries a significant risk of complications and is not suitable for everyone.
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