AL (Light-Chain) Cardiac Amyloidosis: A Review of Diagnosis and Therapy
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- 作者：Rodney H. Falk ; MD<sup>asup><sup> ; sup><sup>rfalk@partners.org" class="auth_mail" title="E-mail the corresponding authorsup> ; Kevin M. Alexander ; MD<sup>asup> ; Ronglih Liao ; PhD<sup>asup> ; Sharmila Dorbala ; MD ; MPH<sup>asup><sup> ; sup><sup>bsup>
- 关键词：cardiomyopathy ; multiple myeloma ; TTR amyloidosis
- 刊名：Journal of the American College of Cardiology
- 出版年：2016
- 出版时间：20 September 2016
- 年：2016
- 卷：68
- 期：12
- 页码：1323-1341
- 全文大小：5365 K
文摘
The amyloidoses are a group of protein-folding disorders in which ≥1 organ is infiltrated by proteinaceous deposits known as amyloid. The deposits are derived from 1 of several amyloidogenic precursor proteins, and the prognosis of the disease is determined both by the organ(s) involved and the type of amyloid. Amyloid involvement of the heart (cardiac amyloidosis) carries the worst prognosis of any involved organ, and light-chain (AL) amyloidosis is the most serious form of the disease. The last decade has seen considerable progress in understanding the amyloidoses. In this review, current and novel approaches to the diagnosis and treatment of cardiac amyloidosis are discussed, with particular reference to AL amyloidosis in the heart.