Duran Mehmet Nuri, Beyazit Fatma, Erbaş Mesut, Özkavak Onur, Acar Celal, Bakirdogen Serkan
Department of Obstetrics and Gynecology, Canakkale Onsekiz Mart University, Canakkale, Turkey.
Department of Anesthesiology, Canakkale Onsekiz Mart University, Canakkale, Turkey.
Obstet Med. 2023 Jun;16(2):130-133. doi: 10.1177/1753495X211019902. Epub 2021 Jun 24.
Pregnancy-associated atypical haemolytic uraemic syndrome is a rare and potentially lethal complement-mediated disorder. It can mimic preeclampsia, gestational hypertension, thrombotic thrombocytopenic purpura and hemolysis, elevated liver enzymes and low platelets syndrome. Thus, it can be hard to distinguish pregnancy-associated atypical haemolytic uraemic syndrome from other causes in peri/post-partum women presenting with features of microangiopathic haemolytic anemia, thrombocytopenia and acute kidney injury. We present a case of a 35-year-old woman in her third pregnancy at 32 weeks' gestation who underwent caesarean section due to fetal distress. She developed severe renal impairment, thrombocytopenia and neurologic symptoms within 24 hours after delivery. A diagnosis of pregnancy-associated atypical haemolytic uraemic syndrome was provided, and treatment with plasma therapy followed by eculizumab was initiated. A rapid improvement of both clinical and laboratory parameters was observed. This case demonstrates the significance of early initiation of anti-complement therapy to prevent irreversible renal damage and possible death in women with pregnancy-associated atypical haemolytic uraemic syndrome.
妊娠相关非典型溶血性尿毒症综合征是一种罕见的、可能致命的补体介导性疾病。它可类似子痫前期、妊娠高血压、血栓性血小板减少性紫癜以及溶血、肝酶升高和血小板减少综合征。因此,对于出现微血管病性溶血性贫血、血小板减少和急性肾损伤特征的围产期/产后女性,很难将妊娠相关非典型溶血性尿毒症综合征与其他病因区分开来。我们报告一例35岁女性,孕32周,第三次妊娠,因胎儿窘迫行剖宫产。产后24小时内她出现了严重的肾功能损害、血小板减少和神经系统症状。诊断为妊娠相关非典型溶血性尿毒症综合征,并开始采用血浆置换治疗,随后使用依库珠单抗。观察到临床和实验室参数迅速改善。该病例表明,对于妊娠相关非典型溶血性尿毒症综合征女性,早期启动抗补体治疗以预防不可逆性肾损伤和可能的死亡具有重要意义。