Musselman Kelsey, Laurence Jeffrey, Magro Cynthia, Rahbari Pasha, Di Vitantonio Thomas, Havryliuk Yelena
Department of Obstetrics and Gynecology, Weill Cornell Medicine, New York, NY, United States of America.
Department of Medicine, Division of Hematology and Medical Oncology, Weill Cornell Medicine, New York, NY, United States of America.
Case Rep Womens Health. 2022 Jun 11;35:e00424. doi: 10.1016/j.crwh.2022.e00424. eCollection 2022 Jul.
Atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy due to inability to regulate the complement cascade, resulting in thrombocytopenia, intravascular hemolysis, and end-organ damage. Over 70% of cases are associated with mutations in complement or complement regulatory proteins, and some two-thirds have recognized complement-activating conditions triggering an aHUS event. We describe a case of aHUS after abdominal myomectomy in a 42-year-old woman that was managed with plasma exchange and eculizumab (an anti-C5 monoclonal antibody). The diagnosis was confirmed by biopsy of normal-appearing deltoid skin that demonstrated microvascular C5b-9 deposition, diagnostic of systemic complement pathway activation. Although extremely uncommon following gynecologic surgery, aHUS should be considered in the setting of postoperative oliguric acute kidney injury, as prompt diagnosis is necessary to prevent significant morbidity and mortality.
非典型溶血性尿毒症综合征（aHUS）是一种罕见的血栓性微血管病，由于无法调节补体级联反应，导致血小板减少、血管内溶血和终末器官损伤。超过70%的病例与补体或补体调节蛋白的突变有关，约三分之二的病例有已知的补体激活情况引发aHUS事件。我们描述了一例42岁女性子宫肌瘤切除术后发生aHUS的病例，该病例采用血浆置换和依库珠单抗（一种抗C5单克隆抗体）进行治疗。通过外观正常的三角肌皮肤活检证实了诊断，活检显示微血管C5b-9沉积，可诊断为系统性补体途径激活。尽管在妇科手术后极为罕见，但在术后少尿性急性肾损伤的情况下应考虑aHUS，因为及时诊断对于预防严重的发病率和死亡率至关重要。
