Macroglossia in Light-Chain Amyloidosis

A 78-year-old man presented to the emergency department with a 2-week history of generalized edema, weakness, and chest pain. He reported that during the past year his tongue had become larger and increasingly stiff and that it had become difficult for him to swallow. Physical examination revealed macroglossia, with indentations from the teeth (Panel A). Laboratory findings were notable for proteinuria (2+ protein on dipstick urinalysis and 505 mg of protein per 24-hour urine collection) and elevated levels of troponin I (1.99 mg per liter [reference range 0.06]) and N-terminal pro-brain natriuretic peptide (58,302 pg per millilter [reference range, 450]).

Transthoracic echocardiography and cardiac magnetic resonance imaging were performed, and the results were consistent with an infiltrative cardiomyopathy. Serum immunoelectrophoresis revealed elevated levels of free lambda light chains (2830 mg per liter; reference range, 8.3 to 27.0).