THE PREMORTEM RECOGNITION OF SYSTEMIC SENILE AMYLOIDOSIS WITH CARDIACINVOLVEMENT

PURPOSE: To recognize systemic senile amyloidosis involving the heart and to determine outcome. PATIENTS AND METHODS: All patients with the diagnosis of amyloidosis at the Mayo Clinic from January 1, 1984 throu gh May 1, 1992, were reviewed. Amyloid was confirmed histologically by sulfated alcian blue and alkaline Congo red staining. The labeled str eptavidin-biotin immunoperoxidase method was used with antisera agains t A kappa, A lambda. AA, transthyretin, and beta(2)-microglobulin. Ant i-P-component and antisera to albumin were used as controls. Chest rad iographs, electrocardiograms, transthoracic echocardiograms, and cardi ac catheterization data of all patients were reviewed. Serum and urine were examined with immunoelectrophoresis and immunofixation for the p resence of a monoclonal protein. Lymphocyte DNA was examined for trans thyretin mutations associated with familial amyloidosis. RESULTS: We i dentified 18 patients with myocardial tissue that stained positive for amyloid with sulfated alcian blue and Congo red and with transthyreti n antisera. Congestive heart failure was present at diagnosis in 17 of the 18 patients. Atrial fibrillation was found in 11 patients. No mon oclonal protein was found in the serum or urine. The echocardiographic findings were consistent with infiltrative cardiomyopathy due to amyl oidosis in 16 patients. Right heart pressures were elevated in all 7 p atients who had right-side heart catheterization. No transthyretin mut ations were found in the leukocyte DNA from 12 patients. The actuarial median survival was 5 years; in contrast, the median survival was 5.4 months in 147 patients with primary amyloidosis (AL) who presented wi th congestive heart failure. CONCLUSION: Patients with cardiac amyloid and no monoclonal protein in the serum or urine must have immunohisto chemical staining for kappa and lambda light chains and transthyretin to distinguish between systemic senile amyloidosis, familial amyloidos is, and AL. Patients with systemic senile amyloidosis should not be tr eated with alkylating agents. Their survival is much longer than that of patients with AL (60 versus 5.4 months).