2/1/23, 9:05 PM The premortem recognition of systemic senile amyloidosis with cardiac involvement - PubMed

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Am J Med. 1996 Oct;101(4):395-400. doi: 10.1016/S0002-9343(96)00229-X.

The premortem recognition of systemic senile

amyloidosis with cardiac involvement
R A Kyle  1 , P C Spittell, M A Gertz, C Y Li, W D Edwards, L J Olson, S N Thibodeau

Affiliations
PMID: 8873510 DOI: 10.1016/S0002-9343(96)00229-X

Abstract
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 through May 1, 1992, were reviewed. Amyloid was confirmed histologically by sulfated alcian
blue and alkaline Congo red staining. The labeled streptavidin-biotin immunoperoxidase method was
used with antisera against A kappa, A lambda, AA, transthyretin, and beta 2-microglobulin. Anti-P-
component and antisera to albumin were used as controls. Chest radiographs, electrocardiograms,
transthoracic echocardiograms, and cardiac catheterization data of all patients were reviewed. Serum
and urine were examined with immunoelectrophoresis and immunofixation for the presence of a
monoclonal protein. Lymphocyte DNA was examined for transthyretin mutations associated with
familial amyloidosis.

Results: We identified 18 patients with myocardial tissue that stained positive for amyloid with
sulfated alcian blue and Congo red and with transthyretin antisera. Congestive heart failure was
present at diagnosis in 17 of the 18 patients. Atrial fibrillation was found in 11 patients. No
monoclonal protein was found in the serum or urine. The echocardiographic findings were consistent
with infiltrative cardiomyopathy due to amyloidosis in 16 patients. Right heart pressures were
elevated in all 7 patients who had right-side heart catheterization. No transthyretin mutations 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 with
congestive heart failure.

Conclusion: Patients with cardiac amyloid and no monoclonal protein in the serum or urine must
have immunohistochemical staining for kappa and lambda light chains and transthyretin to
distinguish between systemic senile amyloidosis, familial amyloidosis, and AL. Patients with systemic
senile amyloidosis should not be treated with alkylating agents. Their survival is much longer than
that of patients with AL (60 versus 5.4 months).

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https://pubmed.ncbi.nlm.nih.gov/8873510/ 1/1