Amyloidosis

Life expectancy may also be affected by the • Amyloid light-chain (AL) amyloidosis • Amyloid A (AA) amyloidosis • Hereditary transthyretin (hATTR) amyloidosis • Wild-type amyloidosis AL Amyloidosis: Treatments and Life Expectancy AL amyloidosis is AL Amyloidosis Treatments If a person's AL amyloidosis is treated effectively within • Alkeran (melphalan) • Cytoxan (cyclophosphamide) • • • AL Life Expectancy The treatments currently available for AL amyloidosis can often help people live longer. In fact, certain drug combinations can sometimes put people with the condition into long-term remission (periods without any symptoms or signs of disease). In a A • Stage 1 — 9.8 years • Stage 2 — 6.3 years • Stage 3 — 5.3 years • Stage 4 — 2.3 years Read more about AA Amyloidosis: Treatments and Life Expectancy • Being advanced in age • Having advanced kidney disease, known as end-stage renal disease • Needing dialysis treatments due to kidney failure • Having high levels of inflammatory proteins in the blood, such as immunoglobulin G or C-reactive protein • Having low levels of serum albumin protein AA Amyloidosis Treatment AA amyloidosis treatment focuses on treating the symptoms of the primary disease and any underlying inflammation, as well as reducing the levels of SAA protein. If these factors can be controlled properly, amyloid deposits made from the misfolded proteins can be reduced and organ function can improve. Decreasing amyloid deposits can help Recent • Enbrel (etanerce...

The two diseases begin the same way – in plasma cells, white Having these two diseases together – multiple myeloma and amyloidosis – can shorten your life expectancy. What Are Multiple Myeloma and Amyloidosis? About 35,000 adults in the United States are diagnosed with MM each year. In multiple myeloma, the cancerous plasma cells grow and multiply. After a while, there’s no room left for the healthy plasma cells. Plasma cells come from the spongy tissue inside your bones, called bone marrow. Plasma cells produce antibodies, which help fight infections. As the cancerous plasma cells grow and outnumber healthy cells, they produce proteins that can be harmful, instead of antibodies. As the disease gets worse, it can cause: • • Confusion, • • Infections more often • More thirst • • Loss of appetite, weight loss • • Weakness, numbness in the legs An early form of the disease – a precancer called The risk of getting multiple melanoma increases as you get older. Black people have a higher risk. And if people in your family have MM, your chances of getting it are higher, too. Amyloidosis Amyloidosis affects fewer than 20,000 people in the U.S. a year. It can affect many parts of the body at once (systemic), or only one part of the body (localized). Amyloidosis can damage tissues in your body. The disease can be serious and even life-threatening if it affects the organs, especially your • • • • Digestive tract • Anyone can get amyloidosis, but up to 70% of people with the disease a...

Amyloidosis is when an abnormal protein called amyloid builds up in your tissues and organs. When it does, it affects their shape and how they work. Amyloidosis is a serious health problem that can lead to life-threatening organ failure. Causes and Types of Amyloidosis Many different proteins can lead to amyloid deposits, but only a few have been linked to major health problems. The type of protein and where it collects tells the type of amyloidosis you have. Amyloid deposits may collect throughout your body or in just one area. The different types of amyloidosis include: AL amyloidosis (immunoglobulin light chain amyloidosis). This is the most common type and used to be called primary amyloidosis. AL stands for “amyloid light chains,” which is the type of protein responsible for the condition. There’s no known cause, but it happens when your bone marrow makes abnormal antibodies that can’t be broken down. It’s linked with a blood cancer called multiple myeloma. It can affect your kidneys, heart, liver, intestines, and nerves. AA amyloidosis. Previously known as secondary amyloidosis, this condition is the result of another chronic infectious or inflammatory disease such as rheumatoid arthritis, Crohn’s disease, or ulcerative colitis. It mostly affects your kidneys, but it can also affect your digestive tract, liver, and heart. AA means the amyloid type A protein causes this type. Dialysis-related amyloidosis (DRA). This is more common in older adults and people who have b...

ON THIS PAGE: You will find out more about body changes and other things that can signal a problem that may need medical care. Use the menu to see other pages. The symptoms of amyloidosis can vary widely, depending on the specific organ or number of organs affected by the buildup of amyloid protein. People with amyloidosis may experience the following symptoms or signs. A symptom is something that only the person experiencing it can identify and describe, such as fatigue, nausea, or pain. A sign is something that other people can identify and measure, such as a fever, rash, or an elevated pulse. Together, signs and symptoms can help describe a medical problem. Sometimes, people with amyloidosis do not have any of the signs and symptoms detailed below. Or, the cause of a symptom may be another medical condition. All of these factors can make diagnosing amyloidosis challenging because the symptoms may be similar to those of numerous other diseases and conditions that are more common than amyloidosis. Symptoms of amyloidosis are usually determined by the organ or function that is affected by the protein buildup. For example: • Kidneys. Amyloidosis in the kidneys will reduce the kidneys’ ability to filter waste and break down proteins. As a result, large amounts of protein may be found in the urine, causing “foamy” urine. The kidneys may even stop working. Decreased urine output and changes in creatinine clearance tests, a blood test to measure kidney function, may be present....

Amyloid fibrils are protein polymers comprising identical monomer units (homopolymers). Functional amyloids play a beneficial role in a variety of physiologic processes (eg, long-term memory formation, gradual release of stored peptide hormones). Amyloidosis results from the accumulation of pathogenic amyloids—most of which are aggregates of misfolded proteins—in a variety of tissues. [ Amyloid Amyloid is defined as in vivo deposited material distinguished by the following: • Historical classification systems Until the early 1970s, the idea of a single amyloid substance predominated. Various descriptive classification systems were proposed based on the organ distribution of amyloid deposits and clinical findings. Most classification systems included primary (ie, in the sense of idiopathic) amyloidosis, in which no associated clinical condition was identified, and secondary amyloidosis, which is associated with chronic inflammatory conditions. Some classification systems included myeloma-associated, familial, and localized amyloidosis. The modern era of amyloidosis classification began in the late 1960s with the development of methods to solubilize amyloid fibrils. These methods permitted chemical amyloid studies. Descriptive terms such as primary amyloidosis, secondary amyloidosis, and others (eg, senile amyloidosis), which are not based on etiology, provide little useful information and are no longer recommended. Modern amyloidosis classification Amyloid is now classified...

• The two kinds of amyloidosis most likely to damage the heart are light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR). • Treatment depends on the specific subtype of amyloidosis. It may include medication (oral, intravenous or subcutaneous), chemotherapy, immunotherapy and organ transplant. What are the symptoms of cardiac amyloidosis? Symptoms of AL and ATTR amyloidosis are determined by the organs involved in the disease. In cardiac amyloidosis, amyloid protein deposits in the heart muscle causes it to become thick and stiffened. Over time, this can cause the heart to become weak. Amyloid protein deposits in the heart can lead to: • Congestive heart failure. Symptoms include shortness of breath during activity or while at rest, fatigue, fluid buildup in the abdomen and legs, and difficulty lying flat at night. • Heart rhythm abnormalities. Symptoms include lightheadedness, dizziness, palpitations, shortness of breath, and fatigue. • Valvular disease. If amyloid deposits in the valves in the heart, this can lead to leaky (regurgitant) or narrowed (stenotic) valvular disease. Specifically, ATTR amyloidosis has been found in some patients being treated for severe aortic stenosis. Symptoms include shortness of breath, exercise intolerance, lightheadedness and dizziness. AL and ATTR amyloidosis can be associated with noncardiac symptoms or diagnoses related to deposits in other parts of the body, including: • Carpal tunnel syndrome (especially bilateral carpal ...

Some types of amyloidosis occur with other diseases. These types may improve with treatment of the other diseases. Some types of amyloidosis may lead to life-threatening organ failure. Treatments may include chemotherapy with strong drugs used to treat cancer. Other types of medications can reduce amyloid production and control symptoms. Some people may benefit from organ or stem cell transplants. Enlarged tongue An enlarged tongue (macroglossia) can be a sign of amyloidosis. It can sometimes also appear rippled along its edge. You may not experience symptoms of amyloidosis until later in the course of the disease. Symptoms may vary, depending on which organs are affected. Signs and symptoms of amyloidosis may include: • Severe fatigue and weakness • Shortness of breath • Numbness, tingling, or pain in the hands or feet • Swelling of the ankles and legs • Diarrhea, possibly with blood, or constipation • An enlarged tongue, which sometimes looks rippled around its edge • Skin changes, such as thickening or easy bruising, and purplish patches around the eyes When to see a doctor See your health care provider if you regularly experience any of the signs or symptoms associated with amyloidosis. Causes There are many different types of amyloidosis. Some types are hereditary. Others are caused by outside factors, such as inflammatory diseases or long-term dialysis. Many types affect multiple organs. Others affect only one part of the body. Types of amyloidosis include: • AL amyl...