Bacteriology and Immunology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
Translational Immunology Research Program, Research Programs' Unit, University of Helsinki, Helsinki, Finland.
Front Immunol. 2020 Apr 3;11:548. doi: 10.3389/fimmu.2020.00548. eCollection 2020.
Preeclampsia is a pregnancy-specific disorder affecting ca 3% of all pregnant women. Preeclampsia is the source of severe pregnancy complications. Later life consequences for mother and infant include increased risk of cardiovascular disease. Preeclampsia is caused by the dysfunction of the endothelium with subsequent activation of complement and coagulation systems. HELLP syndrome is considered to be an extreme complication of preeclampsia but it can also present independently. Diagnostic symptoms in HELLP syndrome are Hemolysis, Elevated Liver enzymes, and Low Platelets. Similar phenotype is present in thrombotic microangiopathies (TMAs) and HELLP syndrome is considered part of the TMA spectrum. Here, we present a case of severe preeclampsia and HELLP syndrome, which exacerbated rapidly and eventually led to need of intensive care, plasma exchange, and hemodialysis. The patient showed signs of hemolysis, disturbance in the coagulation, and organ damage in liver and kidneys. After comprehensive laboratory testing and supportive care, the symptoms did not subside and treatment with complement C5 inhibitor eculizumab was started. Thereafter, the patient started to recover. The patient had pregnancy-induced aHUS. Earlier initiation of eculizumab treatment may potentially shorten and mitigate the disease and hypothetically decrease future health risks of preeclamptic women.
子痫前期是一种妊娠特有的疾病，影响约 3%的孕妇。子痫前期是严重妊娠并发症的根源。母婴的后期生活后果包括心血管疾病风险增加。子痫前期是由内皮功能障碍引起的，随后补体和凝血系统被激活。HELLP 综合征被认为是子痫前期的一种极端并发症，但它也可以独立出现。HELLP 综合征的诊断症状是溶血、肝酶升高和血小板减少。血栓性微血管病（TMA）也存在类似的表型，HELLP 综合征被认为是 TMA 谱的一部分。在这里，我们报告了一例严重的子痫前期和 HELLP 综合征病例，病情迅速恶化，最终需要重症监护、血浆置换和血液透析。患者出现溶血、凝血紊乱以及肝、肾功能损害的迹象。经过全面的实验室检查和支持性治疗，症状并未缓解，开始使用补体 C5 抑制剂依库珠单抗进行治疗。此后，患者开始康复。患者患有妊娠诱导性 aHUS。早期开始使用依库珠单抗治疗可能潜在地缩短和减轻疾病，并假设降低子痫前期妇女未来的健康风险。
