ASPIRIN PROPHYLAXIS IN PAROXYSMAL NOCTURNAL HEMOGLOBINURIA

阵发性睡眠性血红蛋白尿症（PNH）是一种慢性溶血性疾病， 一种以保护膜蛋白丢失为特征的疾病 来自补体介导的溶解的红细胞。与其相关联 主要血栓形成事件的高风险，最常见的是血栓形成， 大的腹内静脉。大多数死于疾病的病人 死于血栓虽然这种倾向的原因是 未知，我们最近发现血小板水平异常 在PNH患者中激活。 我们有一个治疗PNH患者的方案， 剂量口服阿司匹林。本方案旨在体外检测 阿司匹林治疗前后血小板活化的变化 局PNH是一种罕见的疾病，治疗方案包括 几次访问美国国立卫生研究院校园， 月（必须对新鲜样本进行血小板研究）。因为 由于招募患者参与本方案的困难，我们 修改它，使病人更方便。

Paroxysmal nocturnal hemoglobinuria (PNH) is a chronic hemolytic disorder characterized by the loss of membrane proteins that protect erythrocytes from complement mediated lysis. It is associated with a high risk of major thrombotic events, most commonly thrombosis of large intraabdominal veins. Most patients who die of their disease die of thrombosis. While the cause of this predisposition is unknown, we have recently demonstrated abnormal levels of platelet activation in patients with PNH. We have a protocol in place for treating patients with PNH with low dose oral aspirin. This protocol is designed to detect in vitro changes in platelet activation before and after aspirin administration. PNH is a rare disorder, and the protocol involves several visits to the National Institutes of Health campus over a month (platelet studies must be performed on fresh samples). Because of the difficulty of recruiting patients to this protocol, we are amending it to make it more convenient for patients.