Kawano Hiroki, Minagawa Kentaro, Wakahashi Kanako, Kawano Yuko, Sada Akiko, Matsui Toshimitsu, Hirano Hirotaka, Shiomi Hideyuki, Ku Yonson, Katayama Yoshio
Hematology, Department of Medicine, Kobe University Graduate School of Medicine, Japan.
Intern Med. 2012;51(18):2613-6. doi: 10.2169/internalmedicine.51.8202. Epub 2012 Sep 15.
Paroxysmal nocturnal hemoglobinuria (PNH) makes patients susceptible to intravascular hemolysis and thrombosis, and it can be life-threatening in stressful situations. Eculizumab, a humanized monoclonal antibody that inhibits the complement protein C5, has been evaluated as a novel therapy for PNH. We herein describe the case of a 59-year-old Japanese woman with classic PNH, who had been successfully treated with eculizumab, but who later developed acute cholecystitis/cholangitis from gallstones. Although the severe obstructive jaundice requiring endoscopic therapy following cholecystectomy was complicated, critical intravascular hemolysis and thrombosis were not observed. Therefore, utilizing eculizumab during the peri-operative management of PNH patients should be carefully taken into consideration.
阵发性夜间血红蛋白尿（PNH）使患者易发生血管内溶血和血栓形成，在应激情况下可能危及生命。依库珠单抗是一种抑制补体蛋白C5的人源化单克隆抗体，已被评估为治疗PNH的一种新疗法。我们在此描述了一名59岁的日本经典型PNH女性患者，她接受依库珠单抗治疗成功，但后来因胆结石发展为急性胆囊炎/胆管炎。尽管胆囊切除术后出现了需要内镜治疗的严重梗阻性黄疸，但未观察到严重的血管内溶血和血栓形成。因此，在PNH患者围手术期管理中使用依库珠单抗应谨慎考虑。
