Boudes P, Horellou M H, Bletry O, Bendetowicz A V, Nguyen G, Conard J, Vidal E, Godeau P, Samama M
Service de Médecine Interne, Hôpital Pitié-Salpêtrière, Paris.
Ann Med Interne (Paris). 1989;140(1):41-4.
A 45 years old woman with AL amyloidosis presented with a hypofibrinogenemia (fibrinogen 100 mg/dl) without severe bleeding. There was laboratory evidence of fibrinolysis with shortened euglobulin lysis time, decreased alpha-2 plasmin inhibitor and decreased plasminogen. The mechanism of this primary fibrinolysis remains unclear, since there is no enhancement of the tissue-type plasminogen activator. Analysis of the 8 cases related in the literature of excessive fibrinolysis associated with amyloidosis demonstrated improvement of bleeding manifestations and abnormal fibrinolysis following the administration of antifibrinolytic agents.
一名45岁的AL淀粉样变性女性患者出现低纤维蛋白原血症(纤维蛋白原100mg/dl),但无严重出血。实验室检查有纤维蛋白溶解的证据,包括优球蛋白溶解时间缩短、α-2纤溶酶抑制剂减少和纤溶酶原减少。由于组织型纤溶酶原激活物没有增强,这种原发性纤维蛋白溶解的机制仍不清楚。对文献中报道的8例与淀粉样变性相关的过度纤维蛋白溶解病例的分析表明,给予抗纤维蛋白溶解剂后出血表现和异常纤维蛋白溶解得到改善。