重组凝血因子Ⅷ预防治疗血友病A模式的现状及展望  

Status and outlook of prophylactic therapy for haemophilia A with recombinant coagulation factor Ⅷ

作　　者：谢晓艳[1] 孙梅[1] 顾健[1] 

机构地区：[1]江苏省苏北人民医院、扬州市血液学研究所,江苏扬州225001

出　　处：《临床血液学杂志》2016年第5期769-772,共4页Journal of Clinical Hematology

摘　　要：血友病是因凝血因子Ⅷ或Ⅸ缺乏导致的一种遗传性出血性疾病,其中80%-85%的患者为凝血因子Ⅷ缺乏导致的血友病A。严重的血友病与关节自发性出血、残疾和生活质量下降有关,早期持续性的规范治疗是关键。预防治疗通过长期规律性应用凝血因子,与按需治疗（即发生出血后开始治疗）相比,可以防止或减少关节出血次数,维持正常关节及肌肉功能,延缓关节病变进展。Hemophilia is a kind of hereditary hemorrhagic disease caused by the lack of blood clotting factor Ⅷ or Ⅸ,among which 80% to 85% of the patients lack factorⅧinduce hemophilia A.Severe hemophilia A is associated with spontaneous joint bleeding,disability and a decrease in the quality of life,and then early continuous standard treatment is the key.Prophylactic therapy with regular administration of coagulation factor is more beneficial then on-demand treatment（treatment after occurrence of hemorrhage）,by reducing joint bleeding frequency,maintaining the health status of joints and muscle,delaying joint disease progression,thus raising the quality of life.Therefore prophylactic therapy has been the first-line therapy for patients with severe hemophilia A.However,at present,there is no best conclusion about the best plan for prophylactic therapy,such as treatment starting time,dose selection,and when to stop treatment,etc.Based on the latest literatures and research reports,this article reviewed the present situation and future development trend of prophylactic therapy in hemophilia A.

关 键 词：重组凝血因子 血友病A 预防治疗 

分 类 号：R554.1[医药卫生—血液循环系统疾病]