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纤维蛋白溶解增加和淀粉样变性。

Increased fibrinolysis and amyloidosis.

作者信息

Liebman H, Chinowsky M, Valdin J, Kenoyer G, Feinstein D

出版信息

Arch Intern Med. 1983 Apr;143(4):678-82.

PMID:6340622
Abstract

We studied three patients with amyloidosis who had a significant hemorrhagic disorder secondary to increased fibrinolysis. In two of the three patients, bleeding was one of the major initial problems; in the other patient, bleeding occurred later in the course of the disease. All three patients were men with primary or myeloma-associated amyloidosis (ie, immunoglobulin-related or AL). None of the patients had significantly impaired liver function at the time of the diagnosis of increased fibrinolysis. At admission, fibrinogen levels were 60, 170, and 90 mg/dL in the three patients. All three patients had short euglobulin clot lysis times (less than 30 minutes) and evidence of in vitro clot lysis. One patient appeared to respond to aminocaproic acid (EACA). In patients with amyloidosis and bleeding, fibrinolytic tests should be done; in patients with increased fibrinolysis, a diagnosis of amyloidosis should be considered.

摘要

我们研究了三名淀粉样变性患者,他们因纤维蛋白溶解增加而患有严重的出血性疾病。在这三名患者中的两名中,出血是主要的初始问题之一;在另一名患者中,出血发生在疾病后期。所有三名患者均为患有原发性或骨髓瘤相关淀粉样变性(即免疫球蛋白相关或AL)的男性。在诊断纤维蛋白溶解增加时,没有一名患者的肝功能严重受损。入院时,三名患者的纤维蛋白原水平分别为60、170和90mg/dL。所有三名患者的优球蛋白凝块溶解时间均较短(少于30分钟),并有体外凝块溶解的证据。一名患者似乎对氨基己酸(EACA)有反应。对于患有淀粉样变性和出血的患者,应进行纤维蛋白溶解试验;对于纤维蛋白溶解增加的患者,应考虑淀粉样变性的诊断。

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