Systemic Types of Amyloid

2021-02-24 12:00 AM

Primary amyloidosis has amyloid light chain (AL) amyloid, whose fibrillary protein is made of kappa or lambda light chains.


Primary amyloidosis has amyloid light chain (AL) amyloid, whose fibrillary pro-tein is made of kappa or lambda light chains. Primary amyloidosis may be seen in plasma cell disorders (multiple myeloma, B-cell lymphomas, etc.) but most cases occur independent of other diseases.

Reactive systemic amyloidosis (secondary amyloidosis) has amyloid-associatedprotein, whose precursor is serum amyloid A (SAA), an acute-phase reactant produced by the liver which is elevated with ongoing chronic inflammation and neoplasia. Reactive systemic amyloidosis can be seen with a wide variety of chronic diseases, including rheumatoid arthritis, systemic lupus erythematosus, tuberculosis, bronchiectasis, osteomyelitis, inflammatory bowel disease, and cancer.

Familial Mediterranean fever has AA type amyloid with fibrillary protein composedof serum amyloid A (SAA). This autosomal recessive disease is characterized by recurrent inflammation, fever, and neutrophil dysfunction. Gain of function mutations of pyrin are present.

Hemodialysis-associated amyloidosis has Aβ2M type amyloid with precursor protein β2-microglobulin. This form of amyloidosis may cause carpal tunnel syndrome and joint disease.