The following is an excerpt....
BASICS
Description
- A group of diseases characterized by extracellular deposition of insoluble protein fibrils in organs and tissues
- Classification is based on the nature of precursor plasma proteins that form fibril deposits (1):
- Primary (AL): Plasma cell dyscrasia; deposition of protein derived from immunoglobulin light chain fragments
- Secondary or reactive (AA): Complicates chronic infections or inflammatory diseases; deposition of serum amyloid A(SAA) protein
- Heritable or familial (AF): Many different types of variant plasma proteins form amyloid deposits beginning in midlife; most common form is caused by mutations of transthyretin (ATTR)
- Dialysis-related: Deposition of fibrils derived from β2-microglobulin; predilection for osteoarticular structures
- Senile systemic amyloidosis: Deposition of otherwise normal (wild-type) transthyretin in myocardium and other sites ...
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