Rheumatology Unit, Department of Medicine, Faculty of Medical Science, State University of Campinas, São Paulo SP, Brazil.
Semin Arthritis Rheum. 2011 Dec;41(3):517-23. doi: 10.1016/j.semarthrit.2011.05.007. Epub 2011 Aug 24.
To perform a systematic review of the association between antiphospholipid antibodies, antiphospholipid syndrome (APS), and HELLP syndrome (Hemolysis; Elevated Liver enzymes; Low Platelet count), describing clinical features, outcome, pathophysiological findings, and treatment.
We performed a literature search in PubMed using the following MeSH entry terms: HELLP syndrome, anticardiolipin antibodies, lupus anticoagulant, antiphospholipid antibodies, and antiphospholipid syndrome. We limited our search to articles published in the English literature from 1994 to 2010.
We identified 29 case reports/studies including a total of 51 pregnancies with HELLP syndrome in 50 patients. The majority of the cases occurred during the 28 to 36 weeks of pregnancy. Nausea, vomiting, epigastric, or right upper quadrant pain was the most frequently reported symptoms at disease onset. Elevated liver enzymes and low platelet count were reported in all studies. Concomitant hypertension and proteinuria were reported in 2/3 of the patients. Hepatic infarctions were observed in 33.3% pregnancies. However, thrombosis was also reported in the central nervous system, deep or superficial vein thrombosis, skin, intestine, bone, spleen, and adrenal glands. Treatment is still a matter of debate in HELLP syndrome. Aspirin, subcutaneous, intravenous, and oral anticoagulation, and prednisone have been used. In addition to the use of plasma exchange and fresh frozen plasma administration, intravenous immunoglobulins and plasmapheresis have been described.
The incidence of obstetric events in patients with APS is a matter of great interest among rheumatology and gynecology and obstetrics professionals. The current knowledge that antiphospholipid antibodies/APS is not only a thrombotic disease, but also associated with microangiopathic features, can explain the greater prevalence of HELLP syndrome in these patients.
对抗磷脂抗体、抗磷脂综合征（APS）与 HELLP 综合征（溶血；肝酶升高；血小板计数低）之间的相关性进行系统综述，描述其临床表现、结局、病理生理学发现和治疗方法。
我们在 PubMed 中使用以下 MeSH 条目进行文献检索：HELLP 综合征、抗心磷脂抗体、狼疮抗凝剂、抗磷脂抗体和抗磷脂综合征。我们将检索范围限定为 1994 年至 2010 年发表的英文文献。
我们共检索到 29 篇病例报告/研究，其中包括 50 例患者共 51 例 HELLP 综合征妊娠。大多数病例发生在妊娠 28 至 36 周。疾病发作时最常报告的症状是恶心、呕吐、上腹部或右上腹疼痛。所有研究均报告了肝酶升高和血小板计数降低。2/3 的患者报告同时存在高血压和蛋白尿。33.3%的妊娠患者观察到肝梗死。然而，血栓也见于中枢神经系统、深部或浅部静脉血栓、皮肤、肠、骨、脾和肾上腺。HELLP 综合征的治疗仍存在争议。已使用阿司匹林、皮下、静脉和口服抗凝药以及泼尼松。除了使用血浆置换和新鲜冷冻血浆输注外，还描述了静脉注射免疫球蛋白和血浆置换。
抗磷脂抗体/APS 不仅是一种血栓性疾病，还与微血管病特征相关，这一当前认识可以解释这些患者中 HELLP 综合征更为常见。因此，APS 患者产科事件的发生率是风湿病学和妇产学专业人员关注的一个重要问题。
