HELLP综合征合并肝梗死引起产妇死亡一例及文献复习

A Case of Maternal Death Caused by HELLP Syndrome Complicated with Hepatic Infarction and A Review of the Literature

肝梗死是一种极其罕见的致命并发症,与溶血、肝酶升高和低血小板水平为特点的HELLP综合征有关。该病可以由急性肝衰竭发展而来,增加产妇和新生儿死亡率。报告1例38岁女性,既往有妊娠期高血压病史,在妊娠31+3周分娩后发生HELLP综合征。随后发生多器官功能衰竭,尽管用血浆置换以及各种对症治疗,但患者病情逐渐恶化并出现脓毒血症以及休克,随后出现胆酶分离,肝功能逐渐恶化,最后影像学表现为大面积肝梗死,直到分娩后21 d死亡。HELLP综合征合并肝梗死的处理应该是多学科的。使用新鲜冰冻血浆进行血浆置换治疗是有效方法之一,但若肝梗死发展为不可逆性肝损伤,肝移植作为唯一根治性治疗方法,如果适用值得尝试。

Hepatic infarction is an extremely rare and fatal complication associated with HELLP syndrome characterized by hemolysis,elevated liver enzymes,and low platelets.It can develop from acute liver failure and increase maternal and neonatal mortality.A 38-year-old woman with a history of gestational hypertension developed HELLP syndrome after delivery at 31+3 weeks of gestation.Multiple organ failure followed.Despite plasma exchange and symptomatic treatment,the patient′s condition gradually deteriorated with sepsis and shock,followed by biliary enzyme isolation and gradual deterioration of liver function.Finally,imaging findings showed extensive liver infarction and she died 21 days after delivery.The management of HELLP syndrome with hepatic infarction should be multidisciplinary.Plasma exchange using fresh frozen plasma is one of the most effective methods,but if liver infarction progresses to irreversible liver damage,liver transplantation as the only radical treatment,if applicable,is worth trying.