张润花,李登举.重组凝血因子VIIa治疗获得性血友病A合并严重出血的疗效观察[J].内科急危重症杂志,2015,21(4):
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| DOI： |
| 中文关键词: 获得性血友病A 凝血因子VIII抑制物 重组凝血因子VIIa |
| 英文关键词:Acquired Hemophilia A FVIII inhibitor Recombinant activated factor VII |
| 基金项目:湖北省自然科学基金,编号2013CFB075；华中科技大学自主创新研究基金(重点专项项目)编号2014ZHYX023 |
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| 中文摘要: |
| 目的： 提高对获得性血友病A(AHA)的认识及探讨重组凝血因子VIIa(rFVIIa)的使用时机。方法：回顾性分析2例获得性血友病A的患者临床表现、实验室检查、治疗经过及转归。结果：2例患者均以自发性出血为主要临床表现,表现为大面积瘀斑和/或肌肉血肿,病因均不明确。血浆中凝血因子VIII(FVIII)活性下降,可检测到FVIII抑制物。经应用rFVIIa后,严重出血症状可以得到控制,联合免疫抑制剂治疗后,达到临床治愈目的。其中1例患者在疾病反复时及时采用了血浆置换疗法。结论：及时应用rFVIIa控制AHA严重出血,可为应用免疫抑制剂治愈AHA创造时机,复发难治病例可考虑行血浆置换联合免疫抑制剂的治疗措施。 |
| 英文摘要: |
| Objective To further understand acquired hemophilia A and discuss the occasion of using recombinant activated factor VII. Methods Here we retrospectively analyzed the clinical manifestation, laboratory examination, treatment and outcomes of two acquired hemophilia A cases. Results Two patients were with spontaneous hemorrhage which mainly represented as large area of ecchymosis and/or muscle hematoma. But the etiology was not clear. We found that the activity of coagulation factor VIII (FVIII) in plasma was decreased and the FVIII inhibitor was detectable. After applying rFVIIa, severe bleeding symptoms were controlled and clinical cure was also achieved after combining with immunosuppressive treatment. We promptly used plasma replacement therapy in one patient when relapsed. Conclusion If promptly apply rFVIIa in controlling AHA induced bleeding, we may gainStime for applying immunosuppressive agents to cure AHA. For refractory and relapsed cases, plasma exchanging combined with immunosuppressive agents should be considered. |
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