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The premortem recognition of systemic senile amyloidosis with cardiac involvement.
Am J Med. 1996 Oct; 101(4):395-400.AJ

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).

Authors+Show Affiliations

Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

8873510

Citation

Kyle, R A., et al. "The Premortem Recognition of Systemic Senile Amyloidosis With Cardiac Involvement." The American Journal of Medicine, vol. 101, no. 4, 1996, pp. 395-400.
Kyle RA, Spittell PC, Gertz MA, et al. The premortem recognition of systemic senile amyloidosis with cardiac involvement. Am J Med. 1996;101(4):395-400.
Kyle, R. A., Spittell, P. C., Gertz, M. A., Li, C. Y., Edwards, W. D., Olson, L. J., & Thibodeau, S. N. (1996). The premortem recognition of systemic senile amyloidosis with cardiac involvement. The American Journal of Medicine, 101(4), 395-400.
Kyle RA, et al. The Premortem Recognition of Systemic Senile Amyloidosis With Cardiac Involvement. Am J Med. 1996;101(4):395-400. PubMed PMID: 8873510.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The premortem recognition of systemic senile amyloidosis with cardiac involvement. AU - Kyle,R A, AU - Spittell,P C, AU - Gertz,M A, AU - Li,C Y, AU - Edwards,W D, AU - Olson,L J, AU - Thibodeau,S N, PY - 1996/10/1/pubmed PY - 1996/10/1/medline PY - 1996/10/1/entrez SP - 395 EP - 400 JF - The American journal of medicine JO - Am J Med VL - 101 IS - 4 N2 - 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). SN - 0002-9343 UR - https://www.unboundmedicine.com/medline/citation/8873510/The_premortem_recognition_of_systemic_senile_amyloidosis_with_cardiac_involvement_ DB - PRIME DP - Unbound Medicine ER -