Ordi-Ros J, Pérez-Pemán P, Monasterio J
Hospital General Vall d'Hebrón, Medicina Interna 3 Pares, Barcelona, Spain.
Haemostasis. 1994 May-Jun;24(3):165-74. doi: 10.1159/000217098.
Antiphospholipid antibodies (APA) comprise a family of immunoglobulins characterized by their pattern of reactivity in a number of laboratory tests. Included in this family are lupus anticoagulant (LA) anticardiolipin antibodies (ACA) and antibodies causing biologic false positive serologic tests for syphilis (BFP-STS). LA and ACA occur in a variety of conditions, including other autoimmunes disorders, infectious diseases, neoplasic disorders, in association with certain drugs and in otherwise healthy individuals. Clinical interest in LA and ACA is increasing. Antiphospholipid antibody syndrome is characterized by a triad of clinical features which include fetal loss, thromboembolic disease and thrombocytopenia. Other clinical manifestations related with APA are livedo reticularis, cutaneous necrosis, hemolytic anemia, heart valve disease, chorea, migraine and obstetric problems as fetal growth retardation, pre-eclampsia, post-partum serositis or neonatal thrombosis or catastrophic antiphospholipid syndrome. Therapy is mainly directed against the widespread and diverse manifestations associated with the obstruction of small and large vessels. Long-term treatment with oral anticoagulation therapy is advised, even if the venous or arterial occlusion occurred many years previously. In patients with primary antiphospholipid syndrome there is no evidence that the prophylactic administration of steroids or immunosuppression will prevent thromboembolic events. Although the administration of more energetic immunosuppression with cyclophosphamide in pulse form is effective in reducing elevated antibody levels, there is usually a rapid rebound to pretreatment levels shortly after discontinuation of the therapy. A history of recurrent fetal loss requires mandatory treatment during pregnancy. Although the actual prospective risk of pregnancy loss in women with antiphospholipid syndrome and prior pregnancy loss is unknown, it may exceed 60%. Because of this many investigators have treated women with antiphospholipid syndrome with either antiplatelet agents, immunosuppressive agents, or anticoagulants in an attempt to improve pregnancy outcome. Unfortunately, there is no unequivocal proof that any of these therapies are fully efficacious. Despite varying treatment protocols, the live birth rate with treatment was 70%, similar to that reported in the recent randomized clinical trial. Thrombocytopenia and autoimmune hemolytic anemia in patients with APA are treated similarly as patients without APA. Treatment of asymptomatic patients isn't indicated, because only approximately 10-15% of patients with APA developed complications.
抗磷脂抗体（APA）是一类免疫球蛋白，其特征在于在多项实验室检测中的反应模式。该家族包括狼疮抗凝物（LA）、抗心磷脂抗体（ACA）以及导致梅毒生物学假阳性血清学检测（BFP-STS）的抗体。LA和ACA存在于多种情况中，包括其他自身免疫性疾病、传染病、肿瘤性疾病，与某些药物相关以及在其他方面健康的个体中。对LA和ACA的临床关注度正在增加。抗磷脂抗体综合征的特征是具有三联征临床特征，包括胎儿丢失、血栓栓塞性疾病和血小板减少症。与APA相关的其他临床表现有网状青斑、皮肤坏死、溶血性贫血、心脏瓣膜病、舞蹈病、偏头痛以及产科问题，如胎儿生长受限、先兆子痫、产后浆膜炎或新生儿血栓形成或灾难性抗磷脂抗体综合征。治疗主要针对与大小血管阻塞相关的广泛多样的表现。建议采用口服抗凝治疗进行长期治疗，即使静脉或动脉阻塞发生在多年之前。在原发性抗磷脂综合征患者中，没有证据表明预防性使用类固醇或免疫抑制会预防血栓栓塞事件。尽管以脉冲形式使用环磷酰胺进行更积极的免疫抑制可有效降低抗体水平升高，但在治疗中断后不久通常会迅速反弹至治疗前水平。有反复胎儿丢失病史的患者在怀孕期间需要强制治疗。尽管抗磷脂综合征且有既往妊娠丢失史的女性实际的妊娠丢失前瞻性风险尚不清楚，但可能超过60%。因此，许多研究人员用抗血小板药物、免疫抑制剂或抗凝剂治疗抗磷脂综合征女性，试图改善妊娠结局。不幸的是，没有明确证据表明这些疗法中有任何一种是完全有效的。尽管治疗方案各不相同，但治疗后的活产率为70%，与近期随机临床试验报告的相似。APA患者的血小板减少症和自身免疫性溶血性贫血的治疗与无APA患者相似。不建议对无症状患者进行治疗，因为只有约10 - 15%的APA患者会出现并发症。
