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ε-氨基己酸治疗急性早幼粒细胞白血病合并获得性α2-纤溶酶抑制剂缺乏症患者

Epsilon-aminocaproic acid in the treatment of patients with acute promyelocytic leukemia and acquired alpha-2-plasmin inhibitor deficiency.

作者信息

Schwartz B S, Williams E C, Conlan M G, Mosher D F

出版信息

Ann Intern Med. 1986 Dec;105(6):873-7. doi: 10.7326/0003-4819-105-6-873.

Abstract

Patients with acute promyelocytic leukemia often develop bleeding diatheses during treatment. In seven patients who had this disease, the plasma level of alpha-2-plasmin inhibitor was the best predictor of severity of coagulopathy and bleeding. Clinical bleeding occurred when alpha-2-plasmin inhibitor levels measured less than 30% of normal levels. Patients with acute promyelocytic leukemia who had acquired deficiencies of alpha-2-plasmin inhibitor were considered to have deficits similar to those in persons congenitally deficient in alpha-2-plasmin inhibitor, and were assumed to be at increased risk for bleeding. Treatment with the fibrinolytic inhibitor, epsilon-aminocaproic acid, along with heparin resulted in prompt cessation of bleeding, reversal of laboratory evidence of fibrinolysis, and a decreased need for blood product support. The only thrombotic complication--thrombosis around a central venous catheter--resolved when treatment with epsilon-aminocaproic acid was discontinued. Epsilon-aminocaproic acid is a safe and effective therapy for those patients with acute promyelocytic leukemia who develop coagulopathy associated with low levels of alpha-2-plasmin inhibitor.

摘要

急性早幼粒细胞白血病患者在治疗期间常出现出血倾向。在7例患有此病的患者中,α2-纤溶酶抑制剂的血浆水平是凝血病严重程度和出血情况的最佳预测指标。当α2-纤溶酶抑制剂水平低于正常水平的30%时,会发生临床出血。获得性α2-纤溶酶抑制剂缺乏的急性早幼粒细胞白血病患者被认为与先天性α2-纤溶酶抑制剂缺乏者有相似的缺陷,并被认为出血风险增加。纤溶抑制剂ε-氨基己酸与肝素联合治疗可使出血迅速停止,纤溶实验室证据逆转,并减少对血液制品支持的需求。唯一的血栓并发症——中心静脉导管周围血栓形成——在停用ε-氨基己酸治疗后得到缓解。对于那些因α2-纤溶酶抑制剂水平低而发生凝血病的急性早幼粒细胞白血病患者,ε-氨基己酸是一种安全有效的治疗方法。

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