摘要目的：了解原发性抗磷脂抗体综合征合并肺血栓栓塞症的临床特征。方法对近期北京医院收治的1例原发性抗磷脂抗体综合征合并肺血栓栓塞症患者进行分析，并复习32例国内外文献发表的原发性抗磷脂抗体综合征合并肺血栓栓塞症病例。结果33例患者中，男18例，女15例，主要症状包括呼吸困难、胸痛、咯血和下肢疼痛等，其中合并呼吸困难、胸痛、咯血三联征的比例为42.4%，双下肢静脉血栓占38.7%，双侧肺动脉栓塞占87.1%，右房和/或右室增大者占56.3%，合并肺动脉高压者占75.0%。部分患者合并血小板减少，活化部分凝血活酶时间明显延长。及时诊断和治疗能取得良好的疗效。结论原发性抗磷脂抗体综合征合并肺血栓栓塞症患者合并三联征(胸痛、咯血、呼吸困难)的比例高，血栓范围广泛，常合并右房和/或右室扩大、血小板和凝血机制异常，及时诊断和治疗可获得良好的疗效。

abstractsObjective To describe the clinical characteristics of pulmonary thromboembolism (PTE) combining with primary antiphospholipid syndrome (PAPS).Methods We described a case of PAPS combining with PTE and reviewed 32 cases reported in literatures.Results 33 patients (eighteen males and fifteen females) had the most common symptoms such as dyspnea,chest pain,hemoptysis,and lower extremity pain.The ratio of triple syndrome of dyspnea,chest pain,and hemoptysis was 42.4%, while ratios of double lower limbs venous thrombosis and bilateral pulmonary embolism were 38.7% and 87.1%,respectively.The ratio of right atrium and (or) right ventricular enlargement was 56.3%,and that of pulmonary hypertension was 75.0%.The platelet level in some patients was decreased and activated partial thromboplastin time was significantly prolonged.Prompt diagnosis and treatment could achieve good therapeutic effect.Conclusions In patients with PTE combining with PAPS,the incidence rate of PTE triple syndrome is high,and moreover,right atrium and (or) ventricular enlargement, thrombocytopenia and abnormal clotting mechanism,and extensive thrombus are common.Prompt diagnosis and treatment can achieve good therapeutic effect.