ABSTRACT
Myocardial involvement is common in AL amyloidosis and leads to severe heart dysfunction, which is the main prognostic determinant and the cause of death in the great majority of patients. Recently a prognostic index based both on troponins and NT-proBNP has been proposed in AL patients undergoing autologous stem cell transplantation. The molecular mechanisms underlying tissue damage in AL remain undetermined. However, experimental studies suggest that circulating oligomers may be responsible for a substantial proportion of amyloid related toxicity. This chapter investigates the relation between cardiac function, estimated with NT-proBNP, and hematologic response to chemotherapy in AL patients. The reduction of circulating free light chains induced by chemotherapy was immediately paralleled by a decrease of serum NT-proBNP concentration in all patients and by resolution of heart failure in the majority of them, without concomitant reduction of wall thickness at echocardiography.
