Doctor in scrubs


W8MD Diet | COVID-19 portal | Vitamin D | Vaccine | Keto

WikiMD is the world's largest medical encyclopedia with
13,351 pages, 4,235,296 edits & 44,104,384 views.

Free unbiased diet, health and wellness info!

AL amyloidosis

From WikiMD's free health, diet & wellness encyclopedia
Jump to navigation Jump to search
AL amyloidosis
Other namesPrimary systemic amyloidosis (PSA), primary amyloidosis
SpecialtyRheumatology

Amyloid light-chain (AL) amyloidosis, also known as primary amyloidosis, is the most common form of systemic amyloidosis in the US.[1] The disease is caused when a person's antibody-producing cells do not function properly and produce abnormal protein fibers made of components of antibodies called light chains. These light chains come together to form amyloid deposits which can cause serious damage to different organs.[2][3] Abnormal light chains in urine are sometimes referred to as "Bence Jones protein".

Signs and symptoms

AL amyloidosis can affect a wide range of organs, and consequently present with a range of symptoms. The kidneys are the most commonly affected organ in AL amyloidosis. Symptoms of kidney disease and kidney failure can include fluid retention, swelling, and shortness of breath.[4]

In addition to kidneys, AL amyloidosis may affect the heart, peripheral nervous system, gastrointestinal tract, blood, lungs and skin. Heart complications, which affect more than a third of AL patients, include heart failure and irregular heart beat. Other symptoms can include stroke, gastrointestinal disorders, enlarged liver, diminished spleen function, diminished function of the adrenal and other endocrine glands, skin color change or growths, lung problems, bleeding and bruising problems, fatigue, and weight loss.[4][5]

Causes

AL amyloidosis can occur spontaneously. It is, however, often associated with other blood disorders, such as multiple myeloma and Waldenström's macroglobulinemia.[4] About 10% to 15% of patients with multiple myeloma may develop overt AL amyloidosis.[6]

Diagnosis

Both blood and the urine can be tested for the light chains, which may form amyloid deposits, causing disease. However, the diagnosis requires a sample of an affected organ.[4][7]

Treatment

The most effective treatment is autologous bone marrow transplants with stem cell rescues. However many patients are too weak to tolerate this approach.[8][9]

Other treatments can involve application of chemotherapy similar to that used in multiple myeloma.[9] A combination of melphalan and dexamethasone has been found effective in those who are ineligible for stem cell transplantation,[8] and a combination of bortezomib and dexamethasone is now in widespread clinical use.[10][11]

Prognosis

Median survival for patients diagnosed with AL amyloidosis was 13 months in the early 1990s, but had improved to c. 40 months a decade later.[12]

Epidemiology

AL amyloidosis is a rare disease; only 1200 to 3200 new cases are reported each year in the United States. Two thirds of patients with AL amyloidosis are male and less than 5% of patients are under 40 years of age.[4][13]

See also

References

External links

Classification
External resources

External links

Wikipedia
Medicine icon
Medicine icon

This WikiMD article AL amyloidosis is a stub. If you are familiar with the topic AL amyloidosis, you can help us. Paid editors welcome!


 

AL amyloidosis is part of WikiMD's free ^articles!

^AL amyloidosis (article) is provided for informational purposes only. No expressed or implied warranties as to the validity of content.
WikiMD is not a substitute for professional advice. By accessing and using WikiMD you agree to the terms of use.
Templates etc. when imported from Wikipedia, are licensed under CC BY-SA 3.0. See full disclaimers.
W8MD weight loss logo

Ad. Tired of being overweight?. W8MD's physician weight loss program can HELP. Tele medicine available