An unusual case of cardiac amyloidosis.
Cardiac amyloidosis can result from any of the systemic amyloidoses. The disease is often characterized by a restrictive cardiomyopathy although the particular signs and symptoms depend in part on the underlying cause. In addition to managing the symptoms of heart failure, treatment options vary depending on the etiology of amyloid deposition. It is therefore critical to identify the cause of cardiac amyloidosis before initiating definitive therapy. We present a patient with presumed immunoglobulin (AL) amyloidosis who had a circulating lambda monoclonal protein, but a bone marrow biopsy with kappa predominant plasma cells. This unusual finding called into question the diagnosis of AL amyloidosis and highlights the importance and difficulty of determining the cause of cardiac amyloid deposition before initiating treatment. We review the different forms of cardiac amyloidosis and propose a diagnostic algorithm to help identify the etiology of cardiac amyloid deposition before beginning therapy.
Garibaldi
Brian
B
Department of Medicine, The Johns Hopkins Hospital, 601 North Caroline Street, Baltimore, Maryland 21287, USA. bgariba1@jhmi.edu
Zaas
David
D
D000368:Aged; D000682:Amyloid / Q000378:metabolism; D000686:Amyloidosis / Q000175:diagnosis / Q000209:etiology / Q000473:pathology* / Q000628:therapy; D001853:Bone Marrow / Q000276:immunology / Q000473:pathology; D001856:Bone Marrow Examination; D002313:Cardiomyopathy, Restrictive / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D004562:Electrocardiography; D017809:Fatal Outcome; D006801:Humans; D007145:Immunoglobulin kappa-Chains / Q000032:analysis; D008297:Male; D009206:Myocardium / Q000473:pathology*; D010265:Paraproteinemias / Q000150:complications
D000682:Amyloid; D007145:Immunoglobulin kappa-Chains