Kusadokoro Sho, Kimura Naoyuki, Hori Daijiro, Yamaguchi Atsushi
Department of Cardiovascular Surgery, Saitama Medical Center, Jichi Medical University, Saitama, Japan.
Interact Cardiovasc Thorac Surg. 2019 Jun 1;28(6):994-995. doi: 10.1093/icvts/ivz020.
Paroxysmal nocturnal haemoglobinuria is a rare disorder characterized by haemolytic anaemia and pancytopaenia. The use of cardiopulmonary bypass can lead to a haemolytic crisis in patients with paroxysmal nocturnal haemoglobinuria due to activation of complement-mediated haemolysis. We report the successful management of a 69-year-old man undergoing aortic valve replacement with standard heparin-protamine protocol by using eculizumab, a monoclonal antibody of complement factor C5. The surgery was performed without triggering a haemolytic crisis, and the patient was discharged from the hospital without major complications.
阵发性睡眠性血红蛋白尿症是一种罕见的疾病，其特征为溶血性贫血和全血细胞减少。由于补体介导的溶血激活，体外循环的使用可导致阵发性睡眠性血红蛋白尿症患者发生溶血危象。我们报告了一名69岁男性患者成功接受主动脉瓣置换术的病例，该患者采用标准肝素-鱼精蛋白方案，并使用补体因子C5的单克隆抗体依库珠单抗进行治疗。手术未引发溶血危象，患者出院时无重大并发症。
