急性A型主动脉夹层后发生的非典型溶血性尿毒症综合征。
Atypical Hemolytic Uremic Syndrome following Acute Type A Aortic Dissection.
作者信息
Ikushima Eigo, Hisahara Manabu, Nishijima Takuya, Uchiyama Hikaru, Onzuka Tatsushi, Ochiai Yoshie, Muta Tsuyoshi, Tokunaga Shigehiko
机构信息
Department of Cardiovascular Surgery, Japan Community Healthcare Organization (JCHO) Kyushu Hospital, 1-8-1, Kishinoura, Yahatanishi-ku, Kitakyushu, Fukuoka 806-8501, Japan.
Department of Hematology/Oncology, Japan Community Healthcare Organization (JCHO) Kyushu Hospital, 1-8-1, Kishinoura, Yahatanishi-ku, Kitakyushu, Fukuoka 806-8501, Japan.
出版信息
Case Rep Hematol. 2020 Mar 3;2020:2467953. doi: 10.1155/2020/2467953. eCollection 2020.
Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA)-related disease that manifests as a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury (AKI) and is caused by uncontrolled activation of the complement system. We report the case of a 61-year-old woman with acute type A aortic dissection that subsequently developed into aHUS. The hematologic disorders underlying aHUS improved after treatment with the complement inhibitor eculizumab. It is important to consider aHUS when a patient clinically develops a triad of microangiopathic hemolytic anemia, thrombocytopenia, and an increasing creatinine level following cardiovascular surgery.
非典型溶血性尿毒症综合征（aHUS）是一种与血栓性微血管病（TMA）相关的疾病，表现为微血管病性溶血性贫血、血小板减少和急性肾损伤（AKI）三联征，由补体系统的失控激活引起。我们报告了一例61岁女性急性A型主动脉夹层病例，该病例随后发展为aHUS。使用补体抑制剂依库珠单抗治疗后，aHUS潜在的血液系统疾病得到改善。当患者在心血管手术后临床上出现微血管病性溶血性贫血、血小板减少和肌酐水平升高三联征时，考虑aHUS很重要。
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