Abstract

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。

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