Meyer Ashley, Uchida Kathryn, Nguyen Matthew, Vongbunyong Kenny, Ren Dong, Hanna Ramy, Tran Minh-Ha, Darwish Omar
Department of Internal Medicine, University of California Irvine Medical Center, 333 City Blvd West, Suite 500, Orange, CA, 92868, USA.
Department of Pathology and Laboratory Medicine, University of California, Irvine, Orange, CA, USA.
J Med Case Rep. 2024 Dec 26;18(1):610. doi: 10.1186/s13256-024-04804-9.
Thrombotic microangiopathy (TMA) is a rare, life-threatening disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end-organ damage. Atypical hemolytic uremic syndrome (aHUS) is even less common, comprising less than 10% of hemolytic uremic syndrome (HUS) cases. aHUS in postpartum is associated with poor maternal outcomes, with the majority of cases resulting in end-stage renal disease. aHUS, unlike other types of TMA, is related to complement dysregulation. Thus, the current treatment of choice for aHUS is complement blockade, which limits unregulated activation of complement and complement-mediated end organ damage.
We present a rare case of a previously healthy, postpartum, 20-year-old Hispanic female patient who underwent a laparoscopic cholecystectomy and subsequently developed complement-mediated TMA, successfully treated with eculizumab. Unique to our case was renal failure owing to multiple insults and partial resolution of hematologic TMA findings prior to initiation of eculizumab.
Our case emphasizes the importance of clinicians possessing a high degree of clinical awareness and judgement surrounding complement-mediated TMA, aHUS and its subsets, and surgery as a precipitator, regardless of safety, particularly during the postpartum period.
血栓性微血管病（TMA）是一种罕见的、危及生命的疾病，其特征为微血管病性溶血性贫血、血小板减少和终末器官损害。非典型溶血性尿毒症综合征（aHUS）更为少见，占溶血性尿毒症综合征（HUS）病例的比例不到10%。产后aHUS与不良的孕产妇结局相关，大多数病例会发展为终末期肾病。与其他类型的TMA不同，aHUS与补体调节异常有关。因此，目前aHUS的首选治疗方法是补体阻断，这可限制补体的不受控制的激活以及补体介导的终末器官损害。
我们报告了一例罕见病例，一名20岁的西班牙裔健康产后女性患者，在接受腹腔镜胆囊切除术后发生了补体介导的TMA，使用依库珠单抗成功治疗。我们病例的独特之处在于，由于多种损伤导致肾衰竭，并且在开始使用依库珠单抗之前血液学TMA表现部分缓解。
我们的病例强调了临床医生对补体介导的TMA、aHUS及其亚型以及作为诱因的手术（无论安全性如何，尤其是在产后期间）具备高度临床意识和判断力的重要性。
