Senile cardiac amyloidosis with myocardial dysfunction. Diagnosis by endomyocardial biopsy and immunohistochemistry.

Senile cardiac amyloid discovered at autopsy is usually regarded as an incidental finding. However, in immunohistochemical studies of autopsy material, three distinct forms of senile cardiovascular amyloid have been characterized, including a systemic form that diffusely infiltrates the cardiac ventricles. The systemic form can be identified immunohistochemically with use of antiserum to human prealbumin. We diagnosed senile systemic amyloidosis causing cardiac dysfunction in five men (57 to 72 years old) by using antiserum to prealbumin in myocardial biopsy tissue. Clinically, the five patients were indistinguishable from patients with nonsecretory immunoglobulin-derived primary amyloidosis with cardiac involvement; only immunohistochemical staining of myocardial tissue distinguished between the two entities. This distinction is important, because the treatment and prognosis of the two disorders are different. We recommend immunohistochemical staining of myocardial tissue for prealbumin in patients with biopsy-proved cardiac amyloid in whom no monoclonal immunoglobulin light chain is detectable in the serum or urine.

[1]  A. Tajik,et al.  Dynamic left ventricular outflow tract obstruction in cardiac amyloidosis detected by continuous-wave Doppler echocardiography. , 1987, The American journal of cardiology.

[2]  K. Sletten,et al.  Prealbumin: its association with amyloid. , 1987, Journal of clinical pathology.

[3]  H. Leinonen,et al.  Cardiac amyloidosis. Therapeutic and diagnostic difficulties with reference to two different forms of the disease. , 2009, Acta medica Scandinavica.

[4]  P. Eriksson,et al.  Cardiac amyloidosis mimicking hypertrophic cardiomyopathy. , 2009, Acta medica Scandinavica.

[5]  A. Freitas The heart in Portuguese amyloidosis. , 1986 .

[6]  M. Benson Treatment of AL amyloidosis with melphalan, prednisone, and colchicine. , 1986, Arthritis and rheumatism.

[7]  B. Durie,et al.  Primary amyloidosis associated with multiple myeloma. Predictors of successful therapy. , 1986, The American journal of medicine.

[8]  R. Kyle,et al.  Primary systemic amyloidosis. Comparison of melphalan/prednisone versus colchicine. , 1985, The American journal of medicine.

[9]  J. Seward,et al.  Echocardiographic findings in systemic amyloidosis: spectrum of cardiac involvement and relation to survival. , 1985, Journal of the American College of Cardiology.

[10]  K. Sletten,et al.  The amyloid in familial amyloid cardiomyopathy of Danish origin is related to pre-albumin. , 1985, Clinical and experimental immunology.

[11]  L. Yam,et al.  Atlas of cytochemistry & immunochemistry of hematologic neoplasms , 1985 .

[12]  P. Westermark,et al.  Senile systemic amyloidosis. , 1984, The American journal of pathology.

[13]  J. Seward,et al.  Serial echocardiographic observations in patients with primary systemic amyloidosis: an introduction to the concept of early (asymptomatic) amyloid infiltration of the heart. , 1984, Mayo Clinic proceedings.

[14]  R. Kyle,et al.  Clinical significance of histopathologic patterns of cardiac amyloidosis. , 1984, Mayo Clinic proceedings.

[15]  A. Jaffe,et al.  Cardiac amyloidosis simulating hypertrophic cardiomyopathy. , 1984, The American journal of cardiology.

[16]  Kyle Ra,et al.  Amyloidosis (AL). Clinical and laboratory features in 229 cases. , 1983 .

[17]  R A Kyle,et al.  Frequency and distribution of senile cardiovascular amyloid. A clinicopathologic correlation. , 1983, The American journal of medicine.

[18]  P. Westermark,et al.  Senile aortic amyloid. A third distinctive type of age-related cardiovascular amyloid. , 1982, The American journal of pathology.

[19]  P. Westermark,et al.  Senile cardiac amyloid: evidence that fibrils contain a protein immunologically related to prealbumin. , 1981, Immunology.

[20]  K. Sletten,et al.  Senile Cardiac Amyloid is Related to Prealbumin , 1980, Scandinavian journal of immunology.

[21]  P. Westermark,et al.  Senile Cardiac Amyloidosis: Evidence of Two Different Amyloid Substances in the Ageing Heart , 1979 .

[22]  H. Hodkinson,et al.  The clinical significance of senile cardiac amyloidosis: a prospective clinico-pathological study. , 1977, The Quarterly journal of medicine.

[23]  R. Swanton,et al.  Systolic and diastolic ventricular function in cardiac amyloidosis. Studies in six cases diagnosed with endomyocardial biopsy. , 1977, The American journal of cardiology.

[24]  R. Ritchie,et al.  Immunofixation. III. Application to the study of monoclonal proteins. , 1976, Clinical chemistry.

[25]  K. Peterson,et al.  Cardiac amyloidosis, contrictive pericarditis and restrictive cardiomyopathy. , 1976, The American journal of cardiology.

[26]  W. Tourtellotte,et al.  Immunofixation electrophoretic techniques applied to identification of proteins in serum and cerebrospinal fluid. , 1976, Clinical chemistry.

[27]  C. Oakley,et al.  The functional defect in amyloid heart disease. The "stiff heart" syndrome. , 1975, The American journal of cardiology.

[28]  R. Kyle,et al.  Primary amyloidosis and acute leukemia associated with melphalan therapy. , 1974, Blood.

[29]  A. Pomerance Senile cardiac amyloidosis. , 1965, British heart journal.