We describe a 76-year-old Japanese woman with primary systemic amyloidosis who presented with angina pectoris associated with ST-segment and T-wave abnormalities resulting from intramyocardial coronary artery amyloidosis. The patient was admitted to our hospital because of dyspnea and pretibial edema 7 years after the diagnosis of variant angina. A diagnosis of primary systemic amyloidosis (AL amyloid protein) was made after examination of gastric and endomyocardial biopsy specimens. The patient died of progressive, uncontrolled heart failure 3 months later. An autopsy study demonstrated only mild-to-moderate atherosclerosis in the epicardial coronary arteries. However, histological examination of the heart revealed diffuse stenoses and obstructions in the intramural coronary arteries by amyloid deposits. This patient had small-vessel coronary disease with ST-segment changes and angina caused by cardiac amyloidosis. A correct diagnosis of ischemic heart disease due to primary amyloidosis is important for estimation of the prognosis and for appropriate management.
Summary
An autopsy was performed on a 16 years old female who had complained of recurrent attacks of subcutaneous nodules with high fever, and granulocytopenia. Non‐suppurative inflammatory changes were presented in the adipose tissue of the subcutaneous, omenteric, mesenteric, peripancreatic, perirenal, and periadrenal regions. The liver showed enlargement with fatty degeneration. The liver, spleen, and the mesenteric lymph nodes had been invaded with many foamy macrophages and some multinucleated giant cells.
The bone marrow revealed a denned retardation of maturity of granulocytic series by infiltration of macrophages. Under the above findings this case may be included in the type of systemic nodular panniculitis by Steinberg.
Clinical findings : A 10-years-old male. For tow weeks prior to admission he had a cold and sore throat with a temperature of 39°C. Nine days later he noted painful swelling of extremital joints and a rash over both legs and face. This was followed by vomiting, abdominal pain, bloody diarrhea, edema, and origuria. On admission edema was found on the face, hands, and legs. The throat appcraed normal. No abnormality of the heart was apparent. There was painful swelling of almost all joints of the extremities and numerous petechiae were scattered over the extremities and face, in places so extensive. Initial laboratory studies revealed ; moderate anaemia, a slight leucocytosis (12,600) with a normal differential count. Sternal 'puncture was normal, and platelet count was normal (390,000). Rumpel-Leede's test was positive. Bleeding time was slightly, prolonged but the coagulation time was normal.The blood nitrogen was 123.5mg' per lOOcc.There was a slight gamma-globulinaemia, albuminuria and haematuria. Test of occult blood in the stool was positive.
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