Ghazanfar Haider, Nawaz Iqra, Allena Nishant, Ashraf Shoaib, Saad Muhammad, Ali Nisha
Internal Medicine, BronxCare Health System, Bronx, USA.
Internal Medicine/Nephrology, Stony Brook University, Stony Brook, USA.
Cureus. 2022 May 18;14(5):e25096. doi: 10.7759/cureus.25096. eCollection 2022 May.
Atypical hemolytic uremic syndrome (HUS) is a rare but severe form of thrombotic microangiopathies (TMAs) that affects both children and adults. The clinical presentation is usually nonspecific, including a broad spectrum of symptoms ranging from abdominal pain, confusion, diarrhea, fatigue, irritability, hypertension, and lethargy. We present a case of a 36-year-old woman with medical comorbidities of asthma and pulmonary embolism who presented to our hospital in the 36th week of her pregnancy for preterm premature rupture of the membranes. The postoperative course was complicated with a sudden onset drop in hemoglobin and acute onset thrombocytopenia. Complements levels were normal while ADAMTS 13 (von Willebrand factor-cleaving protease) activity was 81% which ruled out ADAMTS 13 deficiency. No significant clinical improvement was seen after five cycles of plasmapheresis. She was later started on Eculizumab biweekly with marked improvement in biochemical and clinical status Prompt diagnosis and treatment of atypical HUS are crucial as the prognosis is poor if untreated. The diagnosis of atypical HUS can be challenging as the classic triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury can be seen in all thrombotic microangiopathies, thus careful clinical and laboratory assessment is required to establish the diagnosis. The new treatment modality, Eculizumab, the anti-complement monoclonal antibody, has become the first-line therapy for treating atypical HUS.
非典型溶血性尿毒症综合征(HUS)是一种罕见但严重的血栓性微血管病(TMA),可累及儿童和成人。其临床表现通常缺乏特异性,包括腹痛、意识模糊、腹泻、疲劳、易怒、高血压和嗜睡等一系列症状。我们报告一例36岁女性病例,她患有哮喘和肺栓塞等合并症,在妊娠第36周因胎膜早破入住我院。术后过程中出现血红蛋白突然下降和急性血小板减少症。补体水平正常,而ADAMTS 13(血管性血友病因子裂解蛋白酶)活性为81%,排除了ADAMTS 13缺乏症。在进行五个疗程的血浆置换后,未观察到明显的临床改善。随后她开始每两周使用依库珠单抗治疗,生化和临床状况有显著改善。非典型HUS的及时诊断和治疗至关重要,因为如果不治疗,预后很差。非典型HUS的诊断可能具有挑战性,因为微血管病性溶血性贫血、血小板减少症和急性肾损伤这一经典三联征可见于所有血栓性微血管病,因此需要仔细的临床和实验室评估来确立诊断。新的治疗方式——抗补体单克隆抗体依库珠单抗,已成为治疗非典型HUS的一线疗法。