抽象的
在慢性血栓栓塞肺动脉高压(CTEPH)中始终观察到血栓性损伤,并在初级肺动脉高压(PPH)中经常发现。然而,它仍然未知,但是,血栓形成是否与抗血栓形成途径的缺陷或先前的血管损伤有关。因此,该研究分析了CTEPH和PPH中遗传性和血栓性危险因素的频率。将作者机构中调查的一百四十七名CTEPH患者进行了比较,与PPH连续99名患者进行了比较。在116例CTEPH患者和83磅患者中,通过免疫学和凝血测定分析磷脂依赖性抗体(抗磷脂抗体和狼疮抗凝血剂)。在1994年以来注册的患者(46 cteph和64磅),还确定了遗传性血栓性危险因素。通过功能测定法测量抗凝血酶,蛋白C和蛋白质S活性。通过聚合酶链反应鉴定因子V和因子II的突变。患有PPH或CTEPH的患者没有增加遗传性血栓性危险因素的患病率。相反,在PPH(10%)中观察到磷脂依赖性抗体的高频率,并且在CTH(20%)中更特别地观察到。 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.