Kashyap K, Hosenpud JD
Division of Cardiovascular Medicine, Medical College of Wisconsin, 9200 W. Wisconsin Ave., Milwaukee, WI 53226, USA.
Curr Treat Options Cardiovasc Med. 1999 Oct;1(3):209-218. doi: 10.1007/s11936-999-0037-1.
No definitive therapy is available for cardiac amyloidosis. Treatment with alkylating agents such as melphalan has improved survival. Patients who do not respond to melphalan have benefited from high-dose chemotherapy with cyclophosphamide followed by peripheral stem cell transplantation. Most patients who have symptomatic cardiac involvement do not survive long enough to complete chemotherapy and therefore may not have the opportunity to show treatment benefit. Early diagnosis and treatment before the organ dysfunction has become irreversible are therefore essential to allow adequate duration of therapy. Lifesaving intervention with cardiac transplantation provides a window of opportunity in which other measures can be instituted.
心脏淀粉样变性尚无确切的治疗方法。使用美法仑等烷化剂进行治疗可提高生存率。对美法仑无反应的患者已从环磷酰胺大剂量化疗及随后的外周干细胞移植中获益。大多数有心脏症状的患者存活时间不足以完成化疗，因此可能没有机会显示出治疗益处。因此，在器官功能障碍变得不可逆转之前进行早期诊断和治疗对于确保足够的治疗疗程至关重要。心脏移植这种挽救生命的干预措施提供了一个机会窗口，在此期间可以采取其他措施。
