Cardiac Amyloidosis

心脏淀粉样变性

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作者:Morie A Gertz

Abstract

Amyloid deposits are defined by their tinctorial properties. Under the light microscope amyloid deposits are eosinophilic and amorphous when stained with hematoxylin and eosin. With Congo red staining the deposits are positive and under polarized light will exhibit green birefringence. Sixty years later electron microscopy demonstrated that all deposits were fibrillar. All amyloid deposits are protein derived. The clinical characteristics will be driven by the nature of the protein subunit. In cardiology, the 2 most common subunits accounting for well more than 90% of cardiac amyloidosis are either immunoglobulin light chain, amyloid light-chain (AL) amyloidosis, or transthyretin; transthyretin (TTR) amyloidosis. Although 70% of patients with systemic amyloidosis have cardiac involvement the diagnosis is made by cardiologists only 20% of the time, suggesting significant gaps in knowledge in how to establish a workflow to arrive at a diagnosis in everyday practice.

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