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目的研究抗磷脂综合征(APS)的临床特点,分析不同时期的APS的分类标准,以提高对这一疾病的认识。方法回顾性分析1996—2006年在仁济医院根据不同时期的分类标准诊断的APS患者的临床和实验室特点。结果1996—2006年满足至少1个分类标准的患者共120例,其中符合1988年Asherson分类标准者有101例,符合1999年Sapporo标准者为96例,符合2006年Sydney标准者为115例。在115例APS患者中,男女比例为1:10.5,平均病程为82.6个月,平均年龄为(41±12)岁。其中90例患者发生血栓事件,以深静脉血栓、脑梗死、皮肤血管为主。92例已婚有生育史的女性患者中,有46例发生病态妊娠。7例患者发生恶性APS。抗心磷脂抗体阳性86例,抗β_2-糖蛋白Ⅰ(β_2-GPⅠ)抗体阳性58例,狼疮抗凝物阳性27例。结论常见的血栓部位为下肢深静脉、脑梗死和皮肤血管。Sydney标准增加抗β_2-GPⅠ抗体作为一项实验室指标,提高了分类标准的敏感性,但是对于一些仅有血小板减少和实验室指标阳性的原发性APS患者的诊断存在局限性。另外,一些针对凝血因子的抗体对APS诊断的意义有待深入研究。
Objective To study the clinical features of anti-phospholipid syndrome (APS) and to analyze the classification criteria of APS in different periods so as to raise awareness of this disease. Methods The clinical and laboratory features of APS patients diagnosed by Renji Hospital from 1996 to 2006 according to different stages of classification were retrospectively analyzed. Results A total of 120 patients met at least one classification criteria from 1996 to 2006, of which 101 patients met the 1988 Asherson classification criteria, 96 patients met the 1999 Sapporo criteria and 115 patients met the 2006 Sydney criteria. In 115 patients with APS, the male-to-female ratio was 1: 10.5 with an average duration of 82.6 months and an average age of (41 ± 12) years. Thrombosis occurred in 90 patients, with deep venous thrombosis, cerebral infarction and cutaneous blood vessels. Of the 92 married women who had a childbearing history, 46 had morbid pregnancies. Seven patients developed malignant APS. Anticancer phospholipid antibody was positive in 86 cases, anti-β_2-GP Ⅰ antibody positive 58 cases, lupus anticoagulant positive in 27 cases. Conclusion Common thrombotic sites are deep veins, cerebral infarction and cutaneous blood vessels. The Sydney standard anti-β_2-GPⅠ antibody was used as a laboratory marker to improve the sensitivity of the taxonomy, but there are limitations in the diagnosis of some primary APS patients with thrombocytopenia and laboratory positives. In addition, some antibodies against clotting factor APS significance of the diagnosis needs further study.