首页> 外文期刊>Amyloid: the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis >Myocardial infarction with 'clean coronaries' caused by amyloid light-chain AL amyloidosis: a case report and literature review.
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Myocardial infarction with 'clean coronaries' caused by amyloid light-chain AL amyloidosis: a case report and literature review.

机译:淀粉样蛋白轻链AL淀粉样变性引起的“清洁冠状动脉”心肌梗死:一例病例报告并文献复习。

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摘要

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.
机译:在AL(淀粉样蛋白轻链)淀粉样变性病中,最大的死亡风险发生在有心脏受累的患者中,这些患者通常会出现舒张功能障碍,然后发生收缩性心力衰竭,易患心律不齐和猝死。在这里,我们提出了心脏淀粉样变性的另一种变化。该患者最近进行了非阻塞性冠状动脉造影,但在干细胞收集和动员后不久就发生了致命的心肌梗塞,准备进行大剂量美法仑和自体干细胞移植(HDM / SCT)的治疗。尸检时发现心脏小血管中淀粉样蛋白广泛沉积,并伴有急性梗死的迹象。心脏淀粉样变性的典型表现是浸润性限制性心肌病,该病例报告和文献综述表明,缺血性小血管淀粉样变性也可能发生。 AL淀粉样变性伴心绞痛的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与治疗相关的并发症。当代测试应旨在检测两种形式的心脏淀粉样变性,这可能会影响治疗和预后。

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