Light chain amyloidosis
Light Chain Amyloidosis
Light chain amyloidosis (AL amyloidosis) is a rare type of amyloidosis—a group of diseases caused by deposits of amyloid proteins in tissues and organs. In light chain amyloidosis, the abnormal proteins in the amyloid deposits are part of the light chain part of an antibody. This disease may affect multiple organs, including the heart, kidney, liver, spleen, lungs, nervous system, and gastrointestinal tract.
The cause of light chain amyloidosis is unknown, but is thought to be related to an abnormal response of plasma cells in the bone marrow to antigens such as viral proteins. This abnormal response results in an increased production of monoclonal protein, which is composed of abnormal light chain proteins. These light chain proteins can form amyloid deposits in tissue and organs.
The symptoms of light chain amyloidosis can vary depending on the organs affected. Common symptoms include fatigue, weight loss, abdominal pain, skin lesions, and swelling of the legs or ankles. Other symptoms may include shortness of breath, chest pain, an irregular heartbeat, confusion, and numbness or tingling in the extremities.
The diagnosis of light chain amyloidosis can be difficult due to the rarity and nonspecific symptoms of the disease. Diagnostic tests may include blood tests, imaging studies (such as MRI or CT scan), biopsy of affected organs, and a urine test to check for amyloid protein deposits. Genetic testing may be used to confirm a diagnosis.
Treatment of light chain amyloidosis may include chemotherapy, radiation therapy, or a stem cell transplant. The goal of treatment is to reduce the production of light chain proteins and to control the symptoms of the disease. In some cases, medications such as steroids or immunosuppressants may be used to control the symptoms.
There is currently no cure for light chain amyloidosis, but treatment can help slow the progression of the disease and improve the patient's quality of life. It is important for those with light chain amyloidosis to follow their physicians' recommendations regarding lifestyle changes, such as getting adequate rest and exercise, and taking medicines as prescribed.
Signs and Symptoms
- Fatigue
- Weight loss
- Abdominal pain
- Skin lesions
- Swelling of the legs or ankles
- Shortness of breath
- Chest pain
- Irregular heartbeat
- Confusion
- Numbness or tingling in the extremities
Diagnosis
- Blood tests
- Imaging studies (such as MRI or CT scan)
- Biopsy of affected organs
- Urine test for amyloid protein deposits
- Genetic testing
Treatment
- Chemotherapy
- Radiation therapy
- Stem cell transplant
- Medications such as steroids or immunosuppressants
- Lifestyle changes such as getting adequate rest and exercise