Jean-Marie Elizabeth M, Cho Jonathan J, Trevino Jose G
Department of Surgery, University of Florida Health Sciences Center.
Department of Anatomy and Cell Biology, College of Medicine, University of Florida, Gainesville, FL, United States.
Medicine (Baltimore). 2020 May 29;99(22):e19731. doi: 10.1097/MD.0000000000019731.
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy defined by the sudden onset of hemolytic anemia, thrombocytopenia, and acute kidney injury (AKI). HUS is categorized as either typical, caused by Shiga toxin-producing Escherichia coli infection, or atypical HUS (aHUS), usually complement mediated or secondary to systemic disease. We describe a rare case of aHUS in an adult male patient with recurrent acute pancreatitis.
A 32-year-old Caucasian male presented to our institution for his third episode of alcohol-induced pancreatitis. He presented with abdominal pain, elevated lipase and pancreatic inflammation on computed tomography consistent with acute pancreatitis. While admitted, he developed sudden onset severe thrombocytopenia, AKI and hemolytic anemia.
DIAGNOSIS, THERAPEUTIC INTERVENTIONS, OUTCOMES: Peripheral blood smear, haptoglobin and hemoglobin level confirmed microangiopathic hemolytic anemia. Worsening anemia, thrombocytopenia and AKI were consistent with the diagnosis of aHUS. The patient's pancreatitis resolved with supportive measures, but resolution of significant thrombocytopenia and AKI was not achieved until administration of eculizumab, a complement inhibiting therapy. Eculizumab therapy provided dramatic improvement in this patient, with platelet count increasing from a low of 11,000 to >100,000 within 48 hours of therapy. Creatinine and hemoglobin levels returned to baseline within 3 weeks.
Recurrent pancreatitis is suggested as the etiology of atypical HUS in this patient and this condition should be recognized and treated in a timely manner for optimal clinical outcomes.
溶血尿毒综合征（HUS）是一种血栓性微血管病，其特征为溶血性贫血、血小板减少和急性肾损伤（AKI）突然发作。HUS分为典型型，由产志贺毒素大肠杆菌感染引起，或非典型HUS（aHUS），通常由补体介导或继发于全身性疾病。我们描述了一例成年男性患者罕见的aHUS病例，该患者患有复发性急性胰腺炎。
一名32岁的白种男性因酒精性胰腺炎第三次发作前来我院就诊。他表现为腹痛、脂肪酶升高以及计算机断层扫描显示的胰腺炎症，符合急性胰腺炎表现。住院期间，他突然出现严重血小板减少、AKI和溶血性贫血。
诊断、治疗干预及结果：外周血涂片、触珠蛋白和血红蛋白水平证实为微血管病性溶血性贫血。贫血、血小板减少和AKI的加重与aHUS的诊断相符。患者的胰腺炎通过支持治疗得以缓解，但直到给予依库珠单抗（一种补体抑制疗法）后，严重血小板减少和AKI才得到缓解。依库珠单抗治疗使该患者病情显著改善，治疗后48小时内血小板计数从低至11,000升至>100,000。肌酐和血红蛋白水平在3周内恢复至基线。
复发性胰腺炎被认为是该患者非典型HUS的病因，应及时识别并治疗这种情况以获得最佳临床结果。
