Dejhansathit Siroj, Suvannasankha Attaya
1 Indiana University School of Medicine, Indianapolis, IN, USA.
J Investig Med High Impact Case Rep. 2019 Jan-Dec;7:2324709619832332. doi: 10.1177/2324709619832332.
Acquired factor X (FX) deficiency is a rare but serious complication of primary amyloidosis, presumably caused by the binding of amyloid proteins to the clotting factors. The prolonged prothrombin time, partial thromboplastin time, and low FX level, which are correctable by mixing study, are the disease hallmarks. An immediate goal of care is to stop bleeding. Clotting factor replacement requires close monitoring of coagulogram and FX levels due to varying FX clearance among patients. High-purity FX is currently approved for hereditary FX deficiency and has been successfully used in some acquired FX deficiency cases. Ongoing bleeding risk complicates the treatment decision. Novel therapies yielding rapid and deep response reduce amyloid protein production and improve long-term outcome.
获得性因子X（FX）缺乏是原发性淀粉样变性的一种罕见但严重的并发症，推测是由淀粉样蛋白与凝血因子结合所致。凝血酶原时间延长、部分凝血活酶时间延长以及FX水平降低，这些通过混合试验可纠正，是该疾病的特征。护理的直接目标是止血。由于患者之间FX清除率不同，凝血因子替代治疗需要密切监测凝血图和FX水平。高纯度FX目前已被批准用于遗传性FX缺乏症，并且已成功用于一些获得性FX缺乏症病例。持续的出血风险使治疗决策复杂化。能产生快速且深度反应的新型疗法可减少淀粉样蛋白生成并改善长期预后。
