非典型血栓性血小板减少性紫癜临床分析

目的:进一步提高对血栓性血小板减少性紫癜(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征。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、经确诊立即应用血浆置换对降低TTP患者的病死率至关重要,联合糖皮质激素及CD20单抗治疗可以进一步降低TTP患者的病死率及复发率。

Clinical Analysis of atypical Thrombotic Thrombocytopenic Purpura

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.