• 非典型血栓性血小板减少性紫癜临床分析
  • Clinical Analysis of atypical Thrombotic Thrombocytopenic Purpura
  • 卢博,刘泽林.非典型血栓性血小板减少性紫癜临床分析[J].内科急危重症杂志,2015,21(3):
    DOI:
    中文关键词:  非典型  血栓性血小板减少性紫癜 临床特点  血浆置换 利妥昔单抗
    英文关键词:atypical  thrombotic thrombocytopenic purpura  clinical characteristic  plasma exchange  rituximab
    基金项目:
    作者单位E-mail
    卢博 深圳市南山区人民医院 lubo_80@126.com 
    刘泽林 深圳市南山区人民医院 liuzelin1962@163.com 
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    中文摘要:
          进一步提高我们对TTP诊疗的认识。方法:回顾性分析我院收治的7例非典型TTP患者的临床资料。结果:7例TTP患者,初诊时均无典型五联征,但均具备血小板减少及微血管病性溶血二联症。诊断确立后予血浆置换联合糖皮质激素,6例有效。1例死亡。2例复发患者予利妥昔单抗治疗,均完全缓解。结论:TTP患者早期快速诊断、一经确立诊断立即应用血浆置换对降低TTP患者的死亡率至关重要,联合糖皮质激素及CD20单抗治疗可以进一步降低TTP患者的死亡率及复发率。
    英文摘要:
          To make a further understanding of diagnose and treatment of TTP. Methods : A retrospective survey of 7 atypical TTP patients confirmed in our hospital. Results : No one of the 7 cases have typical five clinical manifestation at initial stage, but all have thrombocytopenia and microangiopathic hemolytic. The ratio of schistocytes on peripheral blood smear was between 2.0% - 6.7%, with a median ratio of 5%. These patients were treated with a combination therapy of plasma exchange and steroids immidiately once the diagnosis was established. 6 cases had response to treatment, 1 cases was died. Two cases of relapsed patients had been treated with rituximab, both were complete remission. Conclusion: Early detection, rapid diagnosis and immediate treatment with plasma exchange is crucial to reduce mortality in patients with TTP. combination with steroids and CD20 monoclonal antibody may further reduce mortality and relapse rate.