Isolated cardiovascular involvement in light chain amyloidosis

Ahamed Shaheer Ahmed, Sampath Kumar, Gautam Sharma, Sudheer Arava
BMJ Case Reports 2020 April 6, 13 (4)
A 50-year-old woman presented with complaints of palpitations and breathlessness of 6 months' duration. She was being treated elsewhere as a case of dilated cardiomyopathy. On evaluation she had racoon eyes, poor progression of R wave on ECG and low-voltage complexes in the limb leads. Echocardiography revealed biventricular hypertrophy, diastolic dysfunction and moderate systolic dysfunction. Cardiac MRI showed features suggestive of amyloidosis. Bone marrow biopsy revealed raised plasma cell count, and endomyocardial biopsy showed amyloid deposits in the myocardium. Free lambda light chain levels were elevated, even though serum and urine electrophoresis did not show any monoclonal band. In this 'text book case of cardiac amyloidosis', apart from cardiovascular system no other organ system was affected, which is uncommon in primary light chain amyloidosis. The patient was started on CyBorD (cyclophosphamide, bortezomib and dexamethasone) regimen.

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