摘要
Introduction: Cardiac amyloidosis is one of the several systemic manifestations of amyloidosis. It is a relatively uncommon disease. Patients with cardiac amyloidosis clinically present with rapidly progressive heart failure. Case Presentation: We present a case of 64-year-old man with multiple episodes of chest discomfort accompanied sometimes with dyspnoea for the past several months. A non-specific ST-T changes with low voltage limb leads on ECG, a diastolic dysfunction with preserved ejection fraction on echocardiography, increased troponin and BNP, increased serum λ/κ light chain ratio led us to highly suspect amyloidosis with cardiac involvement. Abdominal fat pad biopsy confirmed the amyloid type change of subcutaneous fat, with immunohistochemistry revealing Lambda positive. After confirmed diagnosis of primary systemic amyloidosis with cardiac involvement, patient completed six cycles of chemotherapy with bortezomib and dexamethasone. The patient showed significant improvements after chemotherapy and supportive therapy. Conclusion: Amyloidosis is relatively fatal disease. Patients often have a short life expectancy after symptom onset. Early diagnosis is crucial to proper management and better prognosis.
Introduction: Cardiac amyloidosis is one of the several systemic manifestations of amyloidosis. It is a relatively uncommon disease. Patients with cardiac amyloidosis clinically present with rapidly progressive heart failure. Case Presentation: We present a case of 64-year-old man with multiple episodes of chest discomfort accompanied sometimes with dyspnoea for the past several months. A non-specific ST-T changes with low voltage limb leads on ECG, a diastolic dysfunction with preserved ejection fraction on echocardiography, increased troponin and BNP, increased serum λ/κ light chain ratio led us to highly suspect amyloidosis with cardiac involvement. Abdominal fat pad biopsy confirmed the amyloid type change of subcutaneous fat, with immunohistochemistry revealing Lambda positive. After confirmed diagnosis of primary systemic amyloidosis with cardiac involvement, patient completed six cycles of chemotherapy with bortezomib and dexamethasone. The patient showed significant improvements after chemotherapy and supportive therapy. Conclusion: Amyloidosis is relatively fatal disease. Patients often have a short life expectancy after symptom onset. Early diagnosis is crucial to proper management and better prognosis.
