Amyloid Treatment and Research Program, Boston Medical Center, 820 Harrison Avenue, Boston, MA, USA.
Amyloid. 2011 Sep;18(3):160-4. doi: 10.3109/13506129.2011.571319. Epub 2011 Apr 19.
In AL (amyloid light-chain) amyloidosis, the greatest risk of death occurs in patients with cardiac involvement, who typically develop diastolic dysfunction and then systolic heart failure, with predisposition to arrhythmias and sudden death. Here, we present an alternate variation of cardiac amyloidosis. This patient had recent non-obstructive coronary angiography, yet suffered a fatal myocardial infarction shortly after stem cell collection and mobilization in preparation for treatment with high-dose melphalan and autologous stem cell transplantation (HDM/SCT). On autopsy, widespread deposition of amyloid was found in the small vessels of the heart with evidence of associated acute infarction. While the typical presentation of cardiac amyloidosis is an infiltrating restrictive cardiomyopathy, this case report and literature review illustrate that ischemic small vessel amyloidosis may also occur. Small vessel coronary disease and associated myocardial ischemia should be considered in patients with AL amyloidosis with angina, as its presence may increase treatment-related complications. Contemporary testing should aim to detect both forms of cardiac amyloidosis, which may impact management and prognosis.
在轻链（AL）淀粉样变中，有心脏受累的患者死亡风险最大，他们通常会出现舒张功能障碍，然后是收缩性心力衰竭，易发生心律失常和猝死。在这里，我们介绍一种心脏淀粉样变的变体。该患者最近进行了非阻塞性冠状动脉造影，但在干细胞采集和动员以准备接受高剂量马法兰和自体干细胞移植（HDM/SCT）治疗后不久，发生了致命性心肌梗死。尸检发现，心脏小血管中有广泛的淀粉样物质沉积，并伴有急性梗死的证据。虽然心脏淀粉样变的典型表现为浸润性限制型心肌病，但本病例报告和文献复习表明，缺血性小血管淀粉样变性也可能发生。对于有胸痛的 AL 淀粉样变性患者，应考虑小血管冠状动脉疾病和相关的心肌缺血，因为其存在可能会增加治疗相关的并发症。目前的检测应旨在同时检测这两种形式的心脏淀粉样变，这可能会影响治疗和预后。
