What are the 2 types of amyloidosis?
The most common types of amyloidosis are:
- AL (Primary) Amyloidosis.
- AA (Secondary) Amyloidosis.
- Familial ATTR Amyloidosis.
- Wild-Type (Senile) ATTR Amyloidosis.
What is light chain cardiac amyloidosis?
Amyloid light chain (AL) amyloidosis is a systemic disease characterised by the aggregation of misfolded immunoglobulin light chain (LC), predominantly in the heart and kidneys, causing organ failure. If untreated, the median survival of patients with cardiac AL amyloidosis is 6 months from the onset of heart failure.
Is light chain amyloidosis hereditary?
In the usual type of immunoglobulin light-chain amyloidosis (AL), no hereditary pattern has been identified, and the disease is considered “sporadic.” The only definite requirement for amyloid formation appears to be a plasma cell dyscrasia that produces excess monoclonal amyloid fibril precursor protein.
How can you tell the difference between primary and secondary amyloidosis?
Secondary means it occurs because of another disease or situation. For example, this condition usually occurs due to long-term (chronic) infection or inflammation. In contrast, primary amyloidosis means there is no other disease that is causing the condition.
How long can you live with cardiac amyloidosis?
Amyloidosis has a poor prognosis, and the median survival without treatment is only 13 months. Cardiac involvement has the worst prognosis and results in death in about 6 months after onset of congestive heart failure. Only 5% of the patients with primary amyloidosis survive beyond 10 years.
How do I know what type of amyloidosis I have?
Biopsy. A tissue sample may be taken and checked for signs of amyloidosis. The biopsy may be taken from the fat under the skin on your abdomen (fat aspirate), bone marrow, or an affected organ — such as your liver or kidney. Specialized testing of the tissue can help determine the type of amyloid deposit.
What are the symptoms of light chain disease?
The symptoms of light chain deposition disease include nephrotic syndrome and proteinuria, and high levels of blood lipids along with edema . In case of light-chain deposition disease in the heart, the symptoms often include cardiomegaly, congestive heart failure and irregular heartbeat.
What is a free light chain disease?
Light-chain myeloma, also known as light-chain disease, is a type of multiple myeloma in which the malignant bone marrow cells produce free monoclonal light chain proteins. This type of myeloma is usually more severe than the classic kind.
What causes AL amyloidosis?
The cause of AL amyloidosis is usually a plasma cell dyscrasia, an acquired abnormality of the plasma cell in the bone marrow with production of an abnormal light chain protein (part of an antibody).