Demarmels Biasiutti F, Lämmle B
Hämatologisches Zentrallabor, Universität, Inselspital, Bern.
Ther Umsch. 1992 Dec;49(12):850-8.
The overall incidence per year of deep vein thrombosis is about one per thousand, but may be much higher in the presence of certain clinical risk factors such as advanced age, immobilization, surgical procedures, pregnancy, puerperium, use of oral contraceptive agents and malignancy. Moreover, homocystinuria, nephrotic syndrome, systemic lupus erythematosus and hematological disorders such as paroxysmal nocturnal hemoglobinuria or myeloproliferative syndromes predispose to thrombotic disease. Evaluation of the patient with thromboembolism should include detailed history, clinical examination and laboratory investigation to exclude these secondary thrombophilic states. Primary or hereditary thrombophilia is suspected mainly in patients suffering from (venous) thromboembolism at an early age (< 45 years), especially if recurrent and/or familial thrombosis is present. Hereditary thrombophilia may be due to deficiency of antithrombin III, protein C, protein S or plasminogen, some other defects being less well-established prethrombotic risk factors. These currently recognized primary prethrombotic molecular defects are found in 10 to 30% of patients with idiopathic thromboembolism. In the majority of cases the cause of thrombosis remains unknown.
深静脉血栓形成的年总体发病率约为千分之一，但在存在某些临床风险因素时，如高龄、制动、外科手术、妊娠、产褥期、口服避孕药的使用和恶性肿瘤，发病率可能会高得多。此外，高胱氨酸尿症、肾病综合征、系统性红斑狼疮以及血液系统疾病，如阵发性夜间血红蛋白尿或骨髓增殖性综合征，易引发血栓形成疾病。对血栓栓塞患者的评估应包括详细的病史、临床检查和实验室检查，以排除这些继发性易栓状态。原发性或遗传性易栓症主要在年轻时（<45岁）患有（静脉）血栓栓塞的患者中怀疑，特别是如果存在复发性和/或家族性血栓形成。遗传性易栓症可能是由于抗凝血酶III、蛋白C、蛋白S或纤溶酶原缺乏，其他一些缺陷是不太明确的血栓前危险因素。这些目前公认的原发性血栓前分子缺陷在10%至30%的特发性血栓栓塞患者中发现。在大多数情况下，血栓形成的原因仍然未知。
