Moriyama Makoto, Nagata Takuya, Yoshioka Isaku, Hashimoto Isaya, Matsui Koshi, Okumura Tomoyuki, Tsukada Kazuhiro
Department of Surgery & Science, University of Toyama, 2630 Sugitani, Toyama-city, Toyama 930-0194 Japan.
Surg Case Rep. 2015;1(1):57. doi: 10.1186/s40792-015-0059-8. Epub 2015 Jul 11.
Paroxysmal nocturnal hemoglobinuria (PNH) is acquired hemolytic anemia characterized by symptoms such as anemia and hemoglobinuria. In recent years, eculizumab as an anti-complement (C5) monoclonal antibody has been used for PNH and shown to have marked effects. We performed laparoscopic cholecystectomy in a patient with PNH being treated with eculizumab, and could avoid the risk of perioperative hemolysis and thrombosis. [Patient] The patient was a 48-year-old female who had developed PNH when she was 39 years old. At the age of 46 years, eculizumab administration was initiated once every 2 weeks. During the administration period, neither the progression of anemia nor hemoglobinuria was observed. In March 2013, gallstones were detected, and she was referred to our hospital for surgery. Eculizumab was administered 10 days before surgery, and laparoscopic cholecystectomy was performed in May 2013. After the operation, for the prevention of thrombosis, elastic stockings and a foot pump were used without anticoagulant administration. After the operation, neither the progression of anemia nor hemoglobinuria was observed. On postoperative day 5, eculizumab was administered as planned, and she showed a favorable general condition and was discharged. [Discussion] Perioperative care in PNH patients was conventionally considered to involve a high risk of developing anemia, thrombosis, or infection. However, after the advent of eculizumab, the control of the symptoms of PNH became possible in many patients. In this patient with PNH being treated with eculizumab, safe perioperative management was possible without the development of complications.
阵发性睡眠性血红蛋白尿（PNH）是一种获得性溶血性贫血，其特征为贫血和血红蛋白尿等症状。近年来，依库珠单抗作为一种抗补体（C5）单克隆抗体已被用于治疗PNH，并显示出显著疗效。我们为一名正在接受依库珠单抗治疗的PNH患者实施了腹腔镜胆囊切除术，成功避免了围手术期溶血和血栓形成的风险。[患者] 该患者为48岁女性，39岁时患上PNH。46岁时开始每2周注射一次依库珠单抗。在注射期间，未观察到贫血进展或血红蛋白尿。2013年3月，检测出胆结石，她被转诊至我院接受手术。手术前10天注射了依库珠单抗，并于2013年5月进行了腹腔镜胆囊切除术。术后，为预防血栓形成，使用了弹力袜和足部泵，未进行抗凝剂给药。术后，未观察到贫血进展或血红蛋白尿。术后第5天，按计划注射了依库珠单抗，她的一般状况良好并出院。[讨论] 传统上认为PNH患者的围手术期护理存在发生贫血、血栓形成或感染的高风险。然而自依库珠单抗问世后，许多患者的PNH症状得以控制。对于这名接受依库珠单抗治疗的PNH患者，实现了安全的围手术期管理且未出现并发症。
