Poli Daniela, Gensini Gian Franco
Dipartimento del Cuore e dei Vasi , Azienda Ospedaliero-Universitaria Careggi di Firenze.
Ann Ital Med Int. 2005 Oct-Dec;20(4):218-23.
The antiphospholipid syndrome is associated with complications of pregnancy or venous or arterial thrombosis in the presence of antiphospholipid antibodies. Venous thromboembolism is the most common clinical feature. Pathogenetic mechanisms underlying the syndrome are not completely understood and several hypotheses have been raised. Secondary prophylaxis after venous thromboembolism episodes requires oral anticoagulants (prothrombin time-international normalized ratio 2-3) for 6 to 12 months. More intensive anticoagulant regimens are indicated in case of recurrence. A longer or life-long course of treatment is indicated in patients with recurrent or life-threatening events or in the presence of high titers of antiphospholipid antibodies. Among risk factors for venous thromboembolism, inherited thrombophilia is recently described, such as defects of the physiological coagulation inhibitors, genetic mutations of factor V and factor II of the coagulation cascade and hyperhomocysteinemia. High levels of factor VIII, factor IX and lipoprotein(a) have recently been proposed as new risk factors for venous thromboembolism. The coexistence of inherited thrombophilia seems to enhance the thrombogenic risk, in particular in the venous vascular bed. The new insights into the diagnosis of inherited thrombophilia could allow a better evaluation of the thrombotic risk, leading to tailor the prophylactic strategy.
抗磷脂综合征与抗磷脂抗体存在时的妊娠并发症或静脉或动脉血栓形成有关。静脉血栓栓塞是最常见的临床特征。该综合征的发病机制尚未完全明了，已经提出了几种假说。静脉血栓栓塞发作后的二级预防需要口服抗凝剂（凝血酶原时间-国际标准化比值为2-3），持续6至12个月。复发时需要更强化的抗凝方案。对于复发或危及生命的事件患者或存在高滴度抗磷脂抗体的患者，需要更长疗程或终身治疗。在静脉血栓栓塞的危险因素中，最近描述了遗传性血栓形成倾向，如生理性凝血抑制剂缺陷、凝血级联反应中因子V和因子II的基因突变以及高同型半胱氨酸血症。最近，凝血因子VIII、凝血因子IX和脂蛋白(a)水平升高被认为是静脉血栓栓塞的新危险因素。遗传性血栓形成倾向的共存似乎会增加血栓形成风险，尤其是在静脉血管床。对遗传性血栓形成倾向诊断的新认识可以更好地评估血栓形成风险，从而制定针对性的预防策略。
