The premortem recognition of systemic senile amyloidosis with cardiac involvement

Robert A. Kyle, Peter C. Spittell, Morie Gertz, Chin Yang Li, William D. Edwards, Lyle J. Olson, Stephen N Thibodeau

Research output: Contribution to journalArticle

133 Citations (Scopus)

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 Aκ, Aλ, AA, transthyretin, and β2-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 κ and x 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).

Original languageEnglish (US)
Pages (from-to)395-400
Number of pages6
JournalAmerican Journal of Medicine
Volume101
Issue number4
DOIs
StatePublished - Oct 1996

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Amyloidosis
Prealbumin
Familial Amyloidosis
Amyloid
Immune Sera
Congo Red
Alcian Blue
Urine
Cardiac Catheterization
Survival
Heart Failure
Staining and Labeling
Mutation
Immunoelectrophoresis
Streptavidin
Alkylating Agents
DNA
Biotin
Serum
Cardiomyopathies

ASJC Scopus subject areas

  • Nursing(all)

Cite this

The premortem recognition of systemic senile amyloidosis with cardiac involvement. / Kyle, Robert A.; Spittell, Peter C.; Gertz, Morie; Li, Chin Yang; Edwards, William D.; Olson, Lyle J.; Thibodeau, Stephen N.

In: American Journal of Medicine, Vol. 101, No. 4, 10.1996, p. 395-400.

Research output: Contribution to journalArticle

Kyle, Robert A. ; Spittell, Peter C. ; Gertz, Morie ; Li, Chin Yang ; Edwards, William D. ; Olson, Lyle J. ; Thibodeau, Stephen N. / The premortem recognition of systemic senile amyloidosis with cardiac involvement. In: American Journal of Medicine. 1996 ; Vol. 101, No. 4. pp. 395-400.
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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 Aκ, Aλ, AA, transthyretin, and β2-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 κ and x 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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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 Aκ, Aλ, AA, transthyretin, and β2-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 κ and x 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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