依库珠单抗用于治疗继发于手术侵袭性应激和出血的严重血栓性微血管病。
Eculizumab for Severe Thrombotic Microangiopathy Secondary to Surgical Invasive Stress and Bleeding.
作者信息
Fujita Yoko, Terashita Maho, Yazawa Masahiko, Yamasaki Yukitaka, Imamura Tomonori, Kibayashi Junichiro, Sawai Toshihiro, Hidaka Yoshihiko, Ohtani Katsuki, Inoue Norimitsu, Shibagaki Yugo
机构信息
Division of Nephrology and Hypertension, Department of Internal Medicine, St. Marianna University School of Medicine, Japan.
Department of Infectious Disease, St. Marianna University School of Medicine, Japan.
出版信息
Intern Med. 2020;59(1):93-99. doi: 10.2169/internalmedicine.3315-19. Epub 2020 Jan 1.
Atypical hemolytic uremic syndrome (aHUS) is an extremely rare condition caused by an excessive activation of the complement pathway based on genetic or acquired dysfunctions in complement regulation, leading to thrombotic microangiopathy (TMA). A complement-amplifying condition (CAC) can trigger aHUS occurrence along with complement abnormality. We herein report a case of severe TMA after laparoscopic myomectomy in a healthy woman. This case was eventually diagnosed as complement-mediated TMA secondary to surgical invasive stress as a CAC, with no definitive diagnosis of aHUS despite a genetic test. The patient fully recovered after several eculizumab administrations.
非典型溶血性尿毒症综合征（aHUS）是一种极为罕见的疾病，由补体调节的遗传或后天功能障碍导致补体途径过度激活引起，进而导致血栓性微血管病（TMA）。补体放大状态（CAC）可与补体异常一起触发aHUS的发生。我们在此报告一例健康女性腹腔镜子宫肌瘤切除术后发生严重TMA的病例。该病例最终被诊断为继发于手术侵袭应激作为CAC的补体介导的TMA，尽管进行了基因检测，但未明确诊断为aHUS。患者在多次使用依库珠单抗后完全康复。
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