Immunohistochemical Typing of Tissues from 100 Patients with Systemic Amyloidosis
Tissues obtained at biopsy or autopsy from 267 patients with systemic amyloidosis were evaluated immunologically with a panel of antibodies to specific amyloid fibril proteins. of the first 100 patients classified, Aλ I was found in 26%, Aλ III 10%, Aλ VI 6%, Aκ I 29%, Aκ III 6%, AA 8%, and prealbumin (PA) 15%. of the 15 patients with the PA type, 1 had familial amyloidotic polyneuropathy (FAP) and 8 had a typical history of heart disease associated with senile systemic amyloidosis (SSA). Four others had predominantly neuropathic disease, while the remaining 2 were atypical for FAP or SSA. Atypical clinical features were associated with all amyloid types and serve to emphasize the protean clinical nature of the systemic amyloidoses.
KeywordsFamilial Mediterranean Fever Autonomic Neuropathy Systemic Amyloidosis Familial Amyloidotic Polyneuropathy Amyloid Light Chain
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