Abstract：
Pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) is a rare disease. There are only few reports in the literature, and most are in the puerperium period. It is a thrombotic microangiopathy (TMA) characterized for microangiopathic hemolytic anemia, thrombocytopenia, and renal dysfunction. We report the case of a pregnant patient at 26.3 gestation weeks, who developed clinical features of TMA, neurological alterations, and septic shock; then after fetus and placental delivery, no clinical improvement was observed; a diagnostic protocol was performed due to suspicion of P-aHUS, showing improvement after the plasma exchange sessions and eculizumab. We present here a brief review of the case since it is an entity that needs to be suspected during pregnancy when TMA features and requires an immediate diagnosis to provide timely treatment.
摘要：
妊娠相关非典型溶血性尿毒综合征（P-aHUS）是一种罕见疾病。文献中只有很少的报道，大多数都在产褥期。它是一种血栓性微血管病（TMA），以微血管病性溶血性贫血为特征，血小板减少症,肾功能不全.我们报告了一名妊娠26.3周的孕妇，发展了TMA的临床特征，神经系统改变,和感染性休克；然后在胎儿和胎盘分娩后，未观察到临床改善；由于怀疑P-aHUS而进行了诊断方案，血浆置换和依库珠单抗后显示改善。我们在此简要回顾该病例，因为当TMA特征时，它是需要在怀孕期间怀疑的实体，并且需要立即诊断以提供及时治疗。
