SYSTEMIC TYPES OF AMYLOID
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, tuberculo-sis, 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 recur-rent inflammation, fever, and neutrophil dysfunction.
Gain of function mutations of pyrin are
present.
Hemodialysis-associated
amyloidosis has Aβ2M
type amyloid with precursor pro-tein β2-microglobulin.
This form of amyloidosis may cause carpal tunnel syndrome and joint disease.
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