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系统性淀粉样变性中的血栓栓塞和出血:综述。

Thromboembolism and bleeding in systemic amyloidosis: a review.

机构信息

Cardiology department, Lariboisière Hospital, APHP, 2 rue Ambroise Paré, Paris, France.

Université de Paris, Paris, France.

出版信息

ESC Heart Fail. 2022 Feb;9(1):11-20. doi: 10.1002/ehf2.13701. Epub 2021 Nov 16.

Abstract

The assessment of both thromboembolic and haemorrhagic risks and their management in systemic amyloidosis have been poorly emphasized so far. This narrative review summarizes main evidence from literature with clinical perspective. The rate of thromboembolic events is as high as 5-10% amyloidosis patients, at least in patients with cardiac involvement, with deleterious impact on prognosis. The most known pro-thrombotic factors are heart failure, atrial fibrillation, and atrial myopathy. Atrial fibrillation could occur in 20% to 75% of systemic amyloidosis patients. Cardiac thrombi are frequently observed in patients, particularly in immunoglobulin light chains (AL) amyloidosis, up to 30%, and it is advised to look for them systematically before cardioversion. In AL amyloidosis, nephrotic syndrome and the use of immunomodulatory drugs also favour thrombosis. On the other hand, the bleeding risk increases because of frequent amyloid digestive involvement as well as factor X deficiency, renal failure, and increased risk of dysautonomia-related fall.

摘要

目前,系统性淀粉样变的血栓栓塞和出血风险评估及其管理尚未得到充分重视。本综述从临床角度总结了文献中的主要证据。血栓栓塞事件的发生率高达 5-10%的淀粉样变患者,至少在有心脏受累的患者中,对预后有不良影响。最常见的促血栓形成因素有心衰、房颤和心房心肌病。20%至 75%的系统性淀粉样变患者会发生房颤。心脏血栓在患者中经常被观察到,特别是在免疫球蛋白轻链(AL)淀粉样变中,高达 30%,建议在电复律前系统地寻找这些血栓。在 AL 淀粉样变中,肾病综合征和免疫调节药物的使用也会增加血栓形成的风险。另一方面,由于经常发生淀粉样物质消化累及以及因子 X 缺乏、肾衰竭和与自主神经功能障碍相关的跌倒风险增加,出血风险也会增加。

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