Wieczorek I, MacGregor I R, Prescott R J, Ludlam C A
Department of Haematology, Royal Infirmary of Edinburgh, Scotland.
Blood Coagul Fibrinolysis. 1992 Dec;3(6):823-6. doi: 10.1097/00001721-199212000-00039.
This study was designed to assess whether factors other than high haemoglobin, thrombocytosis and abnormal platelet function predispose to thrombosis in polycythaemia rubra vera (PRV). Components of the fibrinolytic system and concentrations of the naturally occurring anticoagulants were measured in patients and controls in the resting state; the fibrinolytic capacity was reassessed after venous occlusion. The results were related to presence or absence of a history of thromboembolism. Under resting conditions, patients with PRV had reduced plasminogen activator inhibitor antigen levels and higher fibrin plate lysis area and tissue plasminogen activator activity. Protein C, protein S and factor V levels were reduced. Those patients with a history of thromboembolism had decreased tissue plasminogen activator activity after venous occlusion compared to those who had not experienced a thrombosis. We conclude that reduced fibrinolytic capacity may predispose to thrombosis in PRV. Despite treatment to normalize haemoglobin levels, the patients have persistent activation of their fibrinolytic systems. This, and reduced levels of proteins C and S, may be secondary to a chronic, clinically occult, disseminated intravascular coagulation.
本研究旨在评估除高血红蛋白、血小板增多症和异常血小板功能外，其他因素是否易使真性红细胞增多症（PRV）患者发生血栓形成。在静息状态下测量患者及对照者纤溶系统的组成部分和天然抗凝剂的浓度；静脉闭塞后重新评估纤溶能力。结果与有无血栓栓塞病史相关。在静息条件下，PRV患者的纤溶酶原激活物抑制剂抗原水平降低，纤维蛋白平板溶解面积和组织纤溶酶原激活物活性较高。蛋白C、蛋白S和因子V水平降低。与无血栓形成的患者相比，有血栓栓塞病史的患者在静脉闭塞后组织纤溶酶原激活物活性降低。我们得出结论，纤溶能力降低可能易使PRV患者发生血栓形成。尽管进行了使血红蛋白水平正常化的治疗，但患者的纤溶系统仍持续激活。这以及蛋白C和S水平降低可能继发于慢性、临床隐匿的弥散性血管内凝血。
