文摘
血栓性损伤不断地观察慢性血栓栓塞肺动脉高压(CTEPH),经常发现在原发性肺动脉高压(PPH)。仍然是未知的,然而,是否血栓形成与缺陷的抗血栓形成的途径或以前的血管损伤。因此本研究分析了遗传和后天的频率在CTEPH血栓形成的危险因素和产后大出血。一百四十七个连续的患者CTEPH调查99年作者的机构比较连续患者产后大出血。116年,phospholipid-dependent CTEPH患者和83名产后大出血患者抗体(antiphospholipid抗体和狼疮抗凝剂)被免疫和凝血化验分析。自1994年以来,病人登记(46 CTEPH和64年PPH),遗传性血栓形成的危险因素也决定。抗凝血酶,蛋白质C和S活动是衡量功能化验。突变因子V和因子II被确定聚合酶链反应。遗传性血栓形成的危险因素的流行并不增加患者产后大出血或CTEPH。相比之下,高频率的phospholipid-dependent抗体在PPH(10%)和更特别是CTEPH (20%)。 Moreover, in PPH, antibodies were present only in low titre whereas in CTEPH, half of the patients with antiphospholipid antibodies had high titres. In addition, in CTEPH all but one of the patients with lupus anticoagulant also had antiphospholipid antibodies. The most striking finding of this study was the high prevalence of phospholipid-dependent antibodies but their clinical relevance appears to be different in primary pulmonary hypertension and chronic thromboembolic pulmonary hypertension. In primary pulmonary hypertension, these antibodies in low titre probably reflect endothelial dysfunction. In contrast, in chronic thromboembolic pulmonary hypertension the presence of antibodies in high titre associated with lupus anticoagulant, underlines the role of thrombosis in the pathogenesis of this condition.