An abnormal protein called amyloid produced by cells in your bone marrow
|By: John Anderson | Jul 18 2013 | 458 words | 1378 hits|
Amyloidosis is a disease that occurs when substances called amyloid proteins build up in your organs. It is an abnormal protein usually produced by cells in your bone marrow that can be deposited in any tissue or organ. It can affect different organs in different people. It frequently affects the heart, liver, spleen, kidneys, nervous system and gastrointestinal tract.
They depend on which of your organs are affected.
• Swelling of your ankles and legs
• Significant weight loss
• Shortness of breath
• Numbness in your hands or feet
• Diarrhea or constipation
• Feeling full quickly
• Severe fatigue
• An enlarged tongue (macroglossia)
• Skin changes, such as thickening or easy bruising
• Purplish patches (purpura) around the eyes
• An irregular heartbeat
• Difficulty swallowing
• Protein in the urine (detected by urine tests)
• Primary: This most common form can affect many areas, including your intestines, kidneys, heart, spleen, nerves, skin, liver, tongue and blood vessels. It isn't associated with other diseases. The exact cause of it is unknown.
• Secondary: This form occurs in association with chronic infections or inflammatory diseases, such as inflammatory arthritis or osteomyelitis, tuberculosis, a bone infection. It primarily affects your kidneys, liver and lymph nodes, spleen, though other organs may be involved. Treatment of this disease may help stop progression of this form.
• Hereditary (familial): As the name implies, this form is inherited. This type often affects the nerves, liver, kidney and heart.
• Dialysis-related (DRA). This form develops when proteins in blood are deposited in joints and tendons, causing stiffness pain. Risk factors:
• Age: The majority of people who develop this are older than 60.
• Other diseases: You may be at increased risk if you have a chronic infection or inflammatory disease. People who have multiple myeloma — a form of bone marrow cancer — are also at increased risk of this.
• Family history: Some cases of it are inherited.
• Kidney dialysis: If you have kidney disease which requires kidney dialysis, you have an increased risk of it.
• Medicines: Therapies include melphalan (Alkeran) or cyclophosphamide (Cytoxan) — chemotherapy agents that are also used to treat certain types of cancer — and a corticosteroid called dexamethasone is used for its anti-inflammatory effects. These drugs intercept the growth of the abnormal cells that produce amyloid protein.
• Peripheral blood stem cell transplantation: Peripheral blood stem cell transplantation involves using high-dose chemotherapy and transfusion of previously collected immature blood cells (stem cells) to replace diseased or damaged marrow. These cells are your own (autologous transplant).
• Treating secondary amyloidosis: If you have it, the goal of therapy is to treat the underlying condition — for example, taking anti-inflammatory medication for inflammatory arthritis.
• Treating hereditary amyloidosis: For this case, one possible therapy may be liver transplantation because the protein that causes this form is made in the liver.
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