Ornstein M H, Rand J H
Department of Medicine, Mount Sinai School of Medicine, New York, NY 10029.
J Rheumatol. 1994 Jul;21(7):1360-4.
The HELLP syndrome (hemolysis, elevated liver enzymes and low platelets) is a thrombotic microangiopathic vasculopathy that presents in pregnancy. HELLP syndrome usually resolves with delivery of the fetus without sequelae and is often managed successfully with conservative care. To our knowledge, there has been no prior report of an association between HELLP syndrome and elevated titers of antiphospholipid antibodies (aPL). We describe 2 patients who each presented with HELLP syndrome that was refractory despite delivery of the fetus, corticosteroids, and anticoagulation. Both patients had elevated levels of anticardiolipin antibodies. Examination of skin and placental pathology revealed diffuse deposition of fibrin with small vessel thrombi, without evidence of vasculitis. Coincident with plasmapheresis, HELLP resolved in both patients. We conclude that there may be an association between the presence of aPL and a protracted HELLP syndrome in pregnancy; this may be a new manifestation of the antiphospholipid syndrome. Plasmapheresis appears to benefit these patients. Further investigation into this association is warranted.
HELLP综合征（溶血、肝酶升高和血小板减少）是一种发生于妊娠期的血栓性微血管病。HELLP综合征通常在胎儿娩出后缓解，无后遗症，且常通过保守治疗成功处理。据我们所知，此前尚无关于HELLP综合征与抗磷脂抗体（aPL）滴度升高之间关联的报道。我们描述了2例患者，她们均出现了HELLP综合征，尽管已娩出胎儿、使用了皮质类固醇和抗凝治疗，但病情仍难以控制。两名患者的抗心磷脂抗体水平均升高。皮肤和胎盘病理检查显示有纤维蛋白弥漫性沉积及小血管血栓形成，无血管炎证据。在进行血浆置换的同时，两名患者的HELLP综合征均得到缓解。我们得出结论，aPL的存在与妊娠期HELLP综合征迁延不愈之间可能存在关联；这可能是抗磷脂综合征的一种新表现形式。血浆置换似乎对这些患者有益。有必要对这种关联进行进一步研究。
