Griffin G C, Mammen E F, Sokol R J, Perrotta A L, Stoyanovich A, Abildgaard C F
Department of Pediatrics, University of California Davis, Sacramento.
Am J Pediatr Hematol Oncol. 1993 Aug;15(3):328-30.
Alpha 2-Antiplasmin (AP) deficiency is a rare congenital bleeding disorder that presents with normal screening tests for platelet function and clotting. We believe that this disorder is frequently overlooked, especially in women with unexplained bleeding.
We report on two families and one single patient with heterozygous AP deficiency.
All patients and most relatives with the defect had a mild bleeding tendency.
We suggest the incorporation of the AP assay in all patients who have a bleeding disorder with normal platelet studies and normal clotting profiles.
α2 -抗纤溶酶（AP）缺乏症是一种罕见的先天性出血性疾病，其血小板功能和凝血筛查试验结果正常。我们认为这种疾病常被忽视，尤其是在有不明原因出血的女性中。
我们报告了两个家族及一名患有杂合子AP缺乏症的个体。
所有患者及大多数有缺陷的亲属均有轻度出血倾向。
我们建议对所有血小板检查和凝血指标正常但患有出血性疾病的患者进行AP检测。
