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AL amyloidosis is caused by the deposition of abnormal antibody free light chains. The abnormal light chains are produced by monoclonal plasma cells, and, although AL amyloidosis can occur without diagnosis of another disorder, it is often associated with other plasma cell disorders, such as multiple myeloma and Waldenström's macroglobulinemia. [6]
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 ...
Amyloid-forming proteins aggregate into distinctive fibrillar forms with a beta-sheet structure. [19] [20] The beta-sheet form of amyloid is proteolysis-resistant, meaning it can not be degraded or broken down. [5] As a result, amyloid deposits into the body's extracellular space. [5]
Amyloidosis is the most common form of MGRS, with the AL subtype being the most common type. [3] Extra-renal manifestations, such as heart failure, gastrointestinal symptoms, secondary neuropathy (carpal tunnel syndrome), liver involvement and peripheral neuropathy are very common with amyloidosis related MGRS. [3]
Meaning ā (a with a bar over it) before (from Latin ante) before: A: assessment a.a. of each (from Latin ana ana) amino acids: A or Ala – alanine; C or Cys – cysteine; D or Asp – aspartic acid; E or Glu – glutamic acid; F or Phe – phenylalanine; H or His – histidine; I or Ile – isoleucine; K or Lys – lysine; L or Leu ...
Case reports have detected amyloid deposits containing a free γ, α, or μ heavy chain (or portions of one of these chains) accompanied in many cases by a free κ or λ light chain in primarily in the kidney but also the spleen and other tissues. AH plus AHL amyloidosis cases are ~17-fold less common than AL amyloidosis cases. [26]
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