The premortem recognition of systemic senile amyloidosis with cardiac involvement.

摘要: Abstract Purpose To recognize systemic senile amyloidosis involving the heart and to determine outcome. Patients methods All patients with diagnosis of at Mayo Clinic from January 1, 1984 through May 1992, were reviewed. Amyloid was confirmed histologically by sulfated alcian blue alkaline Congo red staining. The labeled streptavidin-biotin immunoperoxidase method used antisera against Aκ, Aλ, Aa, transthyretin, β 2 -microglobulin. Anti-P-component albumin as controls. Chest radiographs, electrocardiograms, transthoracic echocardiograms, cardiac catheterization data all Serum urine examined immunoelectrophoresis immunofixation for presence a monoclonal protein. Lymphocyte DNA transthyretin mutations associated familial amyloidosis. Results We identified 18 myocardial tissue that stained positive amyloid antisera. Congestive failure present in 17 patients. Atrial fibrillation found 11 No protein serum or urine. echocardiographic findings consistent infiltrative cardiomyopathy due 16 Right pressures elevated 7 who had right-side catheterization. leukocyte 12 actuarial median survival 5 years; contrast, 5.4 months 147 primary (AL) presented congestive failure. Conclusion no must have immunohistochemical staining κ λ light chains distinguish between amyloidosis, AL. should not be treated alkylating agents. Their is much longer than AL (60 versus months).