Initial manifestations of light-chain amyloidosis (AL) are variable and often result in missed or delayed diagnosis. Survival in AL patients depends mainly on the severity of cardiac involvement. Dominant stage-III cardiac involvement due to primary systemic amyloidosis precludes effective AL treatment and is associated with an average survival of only 3-4 months. The following paper discusses the benefits of orthotopic heart transplantation and autologous hematopoietic stem cell transplantation to improve survival in patients with progressive cardiac AL.
Keywords: AL amyloidosis; autologous hematopoietic stem cell transplantation; light-chain amyloidosis; orthotopic heart transplantation.