Schafer A I
Ann Intern Med. 1985 Jun;102(6):814-28. doi: 10.7326/0003-4819-102-6-814.
Patients are considered to have hypercoagulable states if they have laboratory abnormalities or clinical conditions that are associated with an increased risk of thrombosis (prethrombotic states) or if they have recurrent thrombosis without recognizable predisposing factors (thrombosis-prone). The number of specific primary hypercoagulable states that are recognized is growing. These disorders are generally inherited abnormalities of coagulation in which a physiologic anticoagulant mechanism is defective: for example, antithrombin III deficiency, protein C and protein S deficiency, abnormalities of the fibrinolytic system, and dysfibrinogenemias. Secondary hypercoagulable states are generally acquired disorders in patients with underlying systemic diseases or clinical conditions known to be associated with an increased risk of thrombosis: for example, malignancy, pregnancy, use of oral contraceptives, myeloproliferative disorders, hyperlipidemia, diabetes mellitus, and abnormalities of blood vessels and rheology. The complex pathophysiologic features of these secondary hypercoagulable states are discussed, and a framework is provided for the laboratory investigation and systematic clinical approach to the patient.
如果患者存在与血栓形成风险增加相关的实验室异常或临床情况(血栓前状态),或者存在无明显易感因素的复发性血栓形成(易栓症),则被认为处于高凝状态。已被确认的特定原发性高凝状态的数量正在增加。这些疾病通常是遗传性凝血异常,其中生理性抗凝机制存在缺陷,例如抗凝血酶III缺乏、蛋白C和蛋白S缺乏、纤维蛋白溶解系统异常以及异常纤维蛋白原血症。继发性高凝状态通常是患有潜在全身性疾病或已知与血栓形成风险增加相关的临床情况的患者所获得的疾病,例如恶性肿瘤、妊娠、口服避孕药的使用、骨髓增殖性疾病、高脂血症、糖尿病以及血管和血液流变学异常。本文讨论了这些继发性高凝状态的复杂病理生理特征,并为患者的实验室检查和系统临床方法提供了一个框架。