中文摘要：

目的研究重组人凝血因子Ⅶa（rFⅦa）治疗血液病患者出血的疗效。方法回顾性分析rFⅦa治疗31例血液病患者共38次出血的临床资料。结果rFⅦa治疗获得性血友病A（AHA）/血友病伴抑制物、急性早幼粒细胞白血病（APL）、急性非APL白血病患者出血的总体有效率分别为90%（9/10）、71.4%（5/7）、60.0%（3/5），高于造血干细胞移植后患者（30.8%）；rFⅦa治疗评分为2分出血的有效率（100.0%）高于3分（66.7%）及4分（51.6%）出血；5例次颅内出血中2例次（25.0%）有效；9例次血尿中6例次（66.7%）有效；12例次消化道出血中5例次（41.7%）有效。3例次关节及肌肉出血的疗效均为极好，5例次出血部位为皮肤、鼻黏膜、咽部及齿龈，疗效均为极好；移植后患者在出血评分为4分时，高剂量以及多次使用rFⅦa并不一定能取得好的疗效。AHA/血友病伴抑制物及急性白血病患者合并的出血，在评分为4分时，使用低剂量rFⅦa能取得好的疗效，但最低用药剂量（22.5 μg/kg）的疗效差。结论rFⅦa的止血疗效受疾病类型、出血部位以及严重程度等多个因素的影响。rFⅦa对AHA/血友病伴抑制物以及急性白血病患者的出血具有较好的疗效，但对移植后患者的出血疗效欠佳。出血早期使用rFⅦa对于疗效至关重要。在止血治疗的同时，应积极治疗基础疾病。

英文摘要：

ObjectiveTo investigate the treatment efficacy of recombinant activated factor Ⅶ （rFⅦa） for bleeding among patients with hematologic disorders.MethodsA total of 38 times of bleeding in 31 patients with hematological disease treated with rFⅦa were analyzed retrospectively.ResultsThe 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.ConclusionsThe 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 hemostati