原位心脏移植促进了轻链淀粉样变性患者的自体造血干细胞移植。
Orthotopic heart transplant facilitated autologous hematopoietic stem cell transplantation in light-chain amyloidosis.
作者信息
Kamble Rammurti T
机构信息
Baylor College of Medicine and The Methodist Hospital, Houston, TX, USA.
出版信息
Methodist Debakey Cardiovasc J. 2012 Jul-Sep;8(3):17-8. doi: 10.14797/mdcj-8-3-17.
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.
轻链淀粉样变性（AL）的初始表现各异，常导致诊断延误或漏诊。AL患者的生存期主要取决于心脏受累的严重程度。原发性系统性淀粉样变性所致的主要Ⅲ期心脏受累会妨碍AL的有效治疗，且平均生存期仅为3 - 4个月。以下论文讨论了原位心脏移植和自体造血干细胞移植对改善进行性心脏AL患者生存期的益处。
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