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~([1])。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~([2]),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,提高 Hemophilia is an inherited hemorrhagic disease caused by deficiency of factor Ⅷ or Ⅸ, of whom 80% to 85% are hemophilia A ~ ([1]) due to lack of blood coagulation factor Ⅷ. Severe hemophilia and spontaneous joint bleeding, disability and quality of life decline ~ ([2]), the early normative treatment is the key to sustained. Prophylaxis By applying clotting factors on a regular basis, it is possible to prevent or reduce the number of joint bleedings, maintain normal joint and muscle function, delay the progression of joint disease, and improve on-demand treatment (ie, treatment started after bleeding occurs)