高三尖酯碱联合阿糖胞苷及G-CSF治疗难治型阵发性睡眠性血红蛋白尿症--附1例报告  

Case report: analysis on the efficaciousness of HAG chemotherapy on treating paroxysmal nocturnal hemoglobinuria

作　　者：成晓敏[1] 麻亮亮[1] 蒋能刚[2] 李建[1] 牛挺[1] 刘霆[1] 贾永前[1] 

机构地区：[1]四川大学华西医院血液内科及血液实验室,四川成都610041 [2]四川大学华西医院实验医学科及流式细胞学检查室,四川成都610041

出　　处：《中国输血杂志》2016年第4期387-390,共4页Chinese Journal of Blood Transfusion

摘　　要：目的探讨难治型阵发性睡眠性血红蛋白尿症(PNH)的有效治疗方案。方法对收治的1名严重输血依赖的难治型PNH伴严重频发血红蛋白尿患者,采用HAG方案[高三尖酯碱(HHT)2 mg/d d1-d7,阿糖胞苷(AraC),12.5 mg/12 h d1-d10,粒细胞集落刺激因子(G-CSF)0.3 mg/d d0-d10)联合化疗治疗2个疗程。结果患者酱油色尿逐渐消失,Hb(g/L)由61升高并维持在110-120,CD55及CD59阴性的红细胞分别从46.8%降至25.3%,51.9%降至23.7%;CD55及CD59阴性的粒细胞分别从80.2%降至59.7%,70.3%降至32.2%。化疗过程耐受良好。结论 HAG方案对激素耐药的PNH患者可能是1个有效的替代治疗手段。Objective To explore efficacious therapeuticplans for refractory paroxysmal nocturnal hemoglobinuria（ PNH）. Methods One patient with refractory PNH received two courses of HAG（ Harringtonine 2mg / d d1- 7; Cytarabine 12. 5 mg /12 hours d1- 10; G-CSF 0. 3 mg / d d0- 10）. Results Patient＇s symptoms gradually eased and her hemoglobin level maintained at 110- 120 g / dl for twelve months without transfusions. Her CD55 and CD59 negative erythrocytes decreased from 46. 8% to 25. 3%,and 51. 9% to 23. 7%,respectively.Her CD55 and CD59 negative granulocytes decreased from 80. 2% to 59. 7%,and 70. 3% to 32. 2%,respectively. She tolerated well during the chemotherapy. Conclusion HAG chemotherapy may be an alternative choice for those patient with prednisone resistance when allogeneic hematopoietic stem cell transplantation and eculizumab are unavailable.

关 键 词：阵发性睡眠性血红蛋白尿症 高三尖脂碱 阿糖胞苷 G-CSF HAG方案 克隆增殖性疾病 

分 类 号：R556.64[医药卫生—血液循环系统疾病] R453[医药卫生—内科学]