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Amyloid light-chain (AL) amyloidosis, also known as primary amyloidosis, is the most common form of systemic amyloidosis. [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 .
AL amyloidosis occurs in about 3–13 per million people per year and AA amyloidosis in about two per million people per year. [2] The usual age of onset of these two types is 55 to 60 years old. [2] Without treatment, life expectancy is between six months and four years. [2]
Light chain deposition disease can affect any organ. [3] Renal involvement is always present and can be identified by microscopic hematuria and proteinuria.Due to the gradual buildup of light chains from plasma filtration, renal function rapidly declines in the majority of patients with LCDD as either acute tubulointerstitial nephritis or rapidly progressing glomerulonephritis.
AA amyloidosis is a form of amyloidosis, a disease characterized by the abnormal deposition of fibers of insoluble protein in the extracellular space of various tissues and organs. In AA amyloidosis, the deposited protein is serum amyloid A protein (SAA), an acute-phase protein which is normally soluble and whose plasma concentration is highest ...
Light chain MGUS is defined as a disorder in which a serum κ to λ free light chain ratio falls outside the normal range of 0.26–1.65 (mean =0.9) provided that it is not associated with: a) any of the CRAB criteria, b) a bone marrow plasma cell count of 10 or a higher percentage of nucleated cells, c) evidence of amyloid deposition (see ...
Light chain (AL-CM) Prognosis: For light-chain amyloidosis early detection leads to best possibility of therapies prolonging the period of remission. [3] Well treated light chain cardiac amyloidosis has a 4-year survival rate of around 90%. [5] In patients that undergo stem cell transplant the average survival time increases to 10 years. [5]
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