Gagne P J, Vitti M J, Fink L M, Duncan J, Nix M L, Barnes R W, Hauer-Jensen M, Barone G W, Eidt J F
Department of Surgery, University of Arkansas for Medical Sciences, Little Rock, USA.
Ann Vasc Surg. 1996 Nov;10(6):546-57. doi: 10.1007/BF02000443.
We identified a group of 24 young (less than 50 years of age) women with isolated, premature atherosclerotic aortoiliac occlusive disease and attempted to identify distinguishing hemostatic characteristics. Most of these patients (62%) presented with acute thromboembolic events (blue toe syndrome, n = 6; macroemboli, n = 6; or aortoiliac thrombosis, n = 3). Aortoiliac reconstruction (aortoiliac endarterectomy, n = 10, aortobifurcation bypass grafts, n = 6; and percutaneous angioplasty, n = 4) was complicated by early thrombosis in 6 of 20 cases (30%), (1 of 10 endarterectomies, 4 of 6 bypass grafts, and 1 of 4 angioplasties). Fresh thrombus overlying an atherosclerotic plaque was a common finding at surgery. This observation and the relatively high incidence of thromboembolic events led us to hypothesize that a characteristic hemostatic profile might underlie the remarkably similar clinical presentations of these women. Levels of antiphospholipid antibodies (anticardiolipin antibodies and lupus anticoagulant), plasminogen activator inhibitor-1, fibrinogen, antithrombin III, protein C, protein S, plasminogen, prothrombin fragment F1 + 2, and D-dimer were determined for these young women and for 21 age-matched white female control subjects without vascular disease and nine white male patients with aortoiliac occlusive disease (mean 61 years, range 43 to 74 years). The incidence of anticardiolipin antibodies was 42% (8 of 19) in the female patients, which was significantly elevated (p = 0.028). The female (62.5%) and male (100%) patients had significantly elevated D-dimer levels (p < 0.001). Deficiencies of antithrombin III, protein C, and protein S were rare. A unique pattern of premature aortoiliac atherosclerosis exists in some young women. Intra-arterial thromboembolic events are common at presentation and complicate surgical management. The role of antiphospholipid antibodies remains uncertain.
我们确定了一组24名年轻（年龄小于50岁）的女性，她们患有孤立性、过早发生的动脉粥样硬化性主髂动脉闭塞性疾病，并试图确定其独特的止血特征。这些患者中的大多数（62%）出现急性血栓栓塞事件（蓝趾综合征，n = 6；大栓子，n = 6；或主髂动脉血栓形成，n = 3）。主髂动脉重建术（主髂动脉内膜切除术，n = 10；主动脉双分叉搭桥术，n = 6；经皮血管成形术，n = 4）在20例中有6例（30%）出现早期血栓形成并发症（10例内膜切除术中的1例，6例搭桥术中的4例，4例血管成形术中的1例）。手术中常见动脉粥样硬化斑块上覆盖新鲜血栓。这一观察结果以及血栓栓塞事件的相对高发生率使我们推测，一种特征性的止血谱可能是这些女性显著相似临床表现的基础。测定了这些年轻女性以及21名无血管疾病的年龄匹配白人女性对照受试者和9名患有主髂动脉闭塞性疾病的白人男性患者（平均61岁，范围43至74岁）的抗磷脂抗体（抗心磷脂抗体和狼疮抗凝物）、纤溶酶原激活物抑制剂-1、纤维蛋白原、抗凝血酶III、蛋白C、蛋白S、纤溶酶原、凝血酶原片段F1 + 2和D-二聚体水平。女性患者中抗心磷脂抗体的发生率为42%（19例中的8例），显著升高（p = 0.028）。女性（62.5%）和男性（100%）患者的D-二聚体水平显著升高（p < 0.001）。抗凝血酶III、蛋白C和蛋白S缺乏症很少见。一些年轻女性存在一种独特的过早发生的主髂动脉粥样硬化模式。动脉内血栓栓塞事件在就诊时很常见，并使手术治疗复杂化。抗磷脂抗体的作用仍不确定。
