心脏淀粉样变性的心脏移植

C. Di Nora, S. Sponga, C. Nalli, M. Driussi, I. Vendramin, G. Benedetti, G. Guzzi, M. Imazio, U. Livi
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引用次数: 0

摘要

已知轻链(AL)或转甲状腺素相关(TTR)淀粉样变患者预后较差。TTR淀粉样变通常比AL淀粉样变进展缓慢,两者都是遗传性TTR淀粉样变(DNA遗传突变)和野生型TTR淀粉样变(通常影响老年人)。本文对目前有关淀粉样变性患者心脏移植的文献进行综述。两种最常见的心脏淀粉样变性被认为是心脏移植的淀粉样变性:AL淀粉样变性和野生型TTR淀粉样变性。根据这一分析,在精心挑选的患者中,心脏移植可能是一个有价值的选择是合理的。此外,它可以改善预后,使AL淀粉样变性亚组的自体干细胞移植成为可能。在我们的拙见中,必须定义一个多学科的方法来帮助选择候选人以获得最有效的结果。
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Heart transplantation in cardiac amyloidosis
It is known that the prognosis of patients affected by light-chain (AL) or transthyretin-related (TTR) amyloidosis is poor. TTR amyloidosis has usually shown a slower progression than AL amyloidosis, both hereditary TTR amyloidosis, where there is an inherited mutation in the DNA, and wild-type TTR amyloidosis, which usually affects the elderly. In this paper, the current literature about heart transplantation on cardiac amyloidosis patients is extensively reviewed. The two most frequent types of cardiac amyloidosis have been considered for heart transplantation: AL amyloidosis and wild-type TTR amyloidosis. According to this analysis, it is reasonable that heart transplantation may represent a valuable option in carefully selected patients. Moreover, it could improve prognosis, enabling autologous stem cell transplantation in the AL amyloidosis subgroup. In our humble opinion, it is mandatory to define a multidisciplinary approach to help select candidates to obtain the most effective results.
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