Systemic lambda light-chain deposition presenting with predominant cardiac involvement

An 82 year old woman with suspected Bence Jones myeloma developed intractable fluid retention presumed secondary to cardiac failure. In addition she experienced angina pectoris, and required permanent cardiac pacing for symptomatic sinus bradycardia. Postmortem studies revealed prominent myocardial and renal deposits of lambda light-chains which were Congo Red negative, and had a non-fibrillar ultrastructure. Non-amyloidotic light-chain deposition is uncommon, and a rare cause of cardiac disease. Previous work regarding possible pathogenetic mechanisms, clinical and laboratory features and treatment is reviewed.