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目的探讨抗磷脂综合征(APS)的临床特征和血清学特点。方法回顾性分析总结100例APS患者的临床表现和免疫学改变。结果原发性APS(PAPS)37例,继发性APS(SAPS)63例,其中继发于系统性红斑狼疮(SLE)46例、狼疮样综合征14例。80%患者出现血管性血栓形成,其中静脉血栓43例、动脉闭塞18例、静脉血栓和动脉闭塞15例。52%为单一部位,32%有2个部位,15%多个部位血栓。56%单次,25%2次,19%多次血栓形成。血栓事件以下肢深静脉血栓(36%)、肺栓塞(30%)和脑卒中(26%)常见。51%(34/67)女性出现病态妊娠,胎死宫内占37%,习惯性自发性流产13%。血小板减少71例。抗心磷脂抗体(AEL)和狼疮抗凝物(IA)的阳性率分别为84%和58%。SLE继发APS组关节炎、白细胞减少、抗核抗体(ANA)阳性和低补体血症较PAPS多见。男性患者下肢深静脉血栓和LA阳性较多见;而女性白细胞减少、ANA阳性和ACL阳性较多见。结论APS以血栓形成及病态妊娠为特点,高滴度ACL和(或)LA阳性是其免疫学特征。其临床表现受基础疾病(SLE)和性别的影响而有不同。
Objective To investigate the clinical features and serological features of antiphospholipid syndrome (APS). Methods Retrospective analysis of 100 cases of APS clinical manifestations and immunological changes. Results There were 37 cases of primary APS (PAPS) and 63 cases of secondary APS (SAPS), including 46 cases of systemic lupus erythematosus (SLE) and 14 cases of lupus-like syndrome. Vascular thrombosis occurred in 80% of patients, including 43 cases of venous thrombus, 18 cases of arterial occlusion, and 15 cases of venous thrombosis and arterial occlusion. 52% for a single site, 32% have 2 sites, more than 15% of thrombosis. 56% single, 25% 2 times, 19% multiple thrombosis. Thrombotic events were common in deep vein thrombosis (36%), pulmonary embolism (30%), and stroke (26%). 51% (34/67) women with morbid pregnancy, intrauterine fetal death accounted for 37%, habitual spontaneous abortion 13%. Thrombocytopenia in 71 cases. The positive rates of AEL and IA were 84% and 58% respectively. SLE secondary APS group arthritis, leukopenia, antinuclear antibody (ANA) positive and hypoacutemia more common than PAPS. Male patients with lower extremity deep venous thrombosis and LA positive more common; and female leukopenia, ANA-positive and ACL-positive more common. Conclusions APS is characterized by thrombosis and morbid pregnancy. High-titer ACL and / or LA-positive immunosuppression is characteristic of APS. Its clinical manifestations vary depending on the underlying disease (SLE) and gender.