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Diagnosis and Classification of Amyloidosis

Subtypes: Historical Context

Slide 5

December 2009

Before the protein constituents of amyloidosis were known, the amyloidosis was classified according to clinical presentation. Those cases with no apparent cause were called primary amyloidosis. We now know that these were mostly AL (light chain) amyloidosis caused by an underlying plasma cell proliferative disorder. The amyloidoses developing secondary to underlying chronic inflammatory disorder such as tuberculosis or rheumatoid arthritis was called secondary amyloidosis. We now know that most of these were caused by abnormal deposition of an acute phase reactant, serum amyloid A (SAA) protein, and are now termed as AA amyloidosis. The other important systemic amyloid type is caused by accumulation by transthyretin or prealbumin, now called ATTR. ATTR amyloidosis can be seen sporadically in advanced age, so-called senile amyloidosis, or as part of a germline mutation affecting the TTR gene, so-called hereditary amyloidosis.

Subtypes: Historical Context


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