Liu W, Xue F, Liu X F, Jiang E L, Yang D L, Liu K Q, Xiao Z J, Zhang F K, Feng S Z, Han M Z, Zhang L, Yang R C
Institute of Hematology & Blood Diseases Hospital, CAMS & PUMC, Tianjin 300020, China.
Zhonghua Xue Ye Xue Za Zhi. 2017 May 14;38(5):410-414. doi: 10.3760/cma.j.issn.0253-2727.2017.05.011.
To investigate the treatment efficacy of recombinant activated factor Ⅶ (rFⅦa) for bleeding among patients with hematologic disorders. A total of 38 times of bleeding in 31 patients with hematological disease treated with rFⅦa were analyzed retrospectively. The clinical effective rate of rFⅦa for bleeding management in acquired hemophilia A (AHA) patients/hemophilia patients with inhibitor, acute promyelocytic leukemia (APL) patients and patients with non-APL leukemia was 90% (9/10) , 71.4% (5/7) and 60.0% (3/5) , respectively, which was higher than that in patients following HSCT (30.8%) . The clinical effective rate of rFⅦa for patients with bleeding score of 2 (100.0%) was higher than that with 3 (66.7%) and 4 (54.1%) . The effective rate of rFⅦa was 25.0% (2/5) in 5 patients with cerebral hemorrhage, 66.7% (6/9) in 9 patients with hematuria and 41.7% in 12 patients with gastrointestinal hemorrhage. The curative effect for 3 patients with joints and muscle bleeding and 5 patients with skin, nasal, pharyngeal and gum bleeding was excellent. Following HSCT, among patients with bleeding score of 4 points, high dose and repeated use of rFⅦa did not necessarily achieve a good effect. Among AHA/hemophilia patients with inhibitors and patients with acute leukemia who had bleeding score of 4 points, the use of low dose FⅦa could achieve good therapeutic effect, however the efficacy of lowest dose (22.5 μg/kg) rFⅦa was poor. The hemostasis efficacy of rFⅦa is affected by various factors such as diseases, bleeding sites, bleeding score and so on. The use of rFⅦa can achieve good efficacy for bleeding management in AHA patients/hemophilia patients with inhibitor, APL patients and patients with non-APL leukemia. However the efficacy of rFⅦa for bleeding of patients after HSCT is poor. Early use of rFⅦa is important for successful hemostatic treatment. Management of underlying condition is as important as hemostatic treatment.
探讨重组活化因子Ⅶ（rFⅦa）治疗血液系统疾病患者出血的疗效。回顾性分析31例接受rFⅦa治疗的血液系统疾病患者共38次出血情况。rFⅦa对获得性血友病A（AHA）患者/有抑制物的血友病患者、急性早幼粒细胞白血病（APL）患者及非APL白血病患者出血的临床有效率分别为90%（9/10）、71.4%（5/7）和60.0%（3/5），高于异基因造血干细胞移植（HSCT）后患者（30.8%）。rFⅦa对出血评分为2分的患者临床有效率（100.0%）高于评分为3分（66.7%）和4分（54.1%）的患者。rFⅦa对5例脑出血患者的有效率为25.0%（2/5），对9例血尿患者的有效率为66.7%（6/9），对12例胃肠道出血患者的有效率为41.7%。对3例关节和肌肉出血患者及5例皮肤、鼻、咽和牙龈出血患者疗效极佳。HSCT后，出血评分为4分的患者中，高剂量及重复使用rFⅦa不一定能取得良好效果。在AHA/有抑制物的血友病患者及出血评分为4分的急性白血病患者中，使用低剂量FⅦa可取得良好治疗效果，但最低剂量（22.5μg/kg）rFⅦa疗效较差。rFⅦa的止血疗效受疾病、出血部位、出血评分等多种因素影响。rFⅦa用于AHA患者/有抑制物的血友病患者、APL患者及非APL白血病患者出血的治疗可取得良好疗效。但rFⅦa对HSCT后患者出血的疗效较差。早期使用rFⅦa对止血治疗成功很重要。治疗基础疾病与止血治疗同样重要。
