继发性血栓性血小板减少性紫癜17例临床分析  

Secondary Thrombotic Thrombocytopenic Purpura:Clinical Analysis of 19 Cases

作　　者：辛鹏亮[1] 李纯团[1] 刘德斌[1] 许文前[1] 肖慧芳[1] 黄远玲[1] 朱雄鹏[1] 

机构地区：[1]福建医科大学附属泉州第一医院血液科,泉州362000

出　　处：《血栓与止血学》2014年第5期235-237,242,共4页Chinese Journal of Thrombosis and Hemostasis

摘　　要：目的 总结分析继发性血栓性血小板减少性紫癜（继发性TTP）患者的临床表现、实验室特点及治疗,以提高对继发性TTP的诊断和治疗水平.方法 对我院2003年1月至2013年12月期间诊断为继发性TTP的17例患者的临床特点和治疗转归进行分析.结果 17例患者中10例（58.82％）出现三联征（血小板减少、微血管病性溶血性贫血、神经系统症状）;6例（35.29％）于病程中出现典型TTP五联征（发热、血小板减少、微血管病性溶血性贫血、神经系统症状和肾脏损害）.本组患者外周血涂片均可见破碎红细胞,范围为1.5％ ～16.5％.本组患者的治疗以血浆置换和输注血浆为主,联用糖皮质激素等免疫抑制剂,治疗有效率64.71％.结论 继发性TTP的诊断主要依赖临床特点,破碎红细胞比例＞1％作为诊断指标有助于早期诊断、早期治疗;不同原因继发性TTP预后不同,应积极处理原发病或消除诱导TTP发作的诱因,尽早给予血浆治疗,并联用糖皮质激素等免疫抑制剂以提高疗效、降低死亡率.Objective To investigate clinical features,out come and laboratory characteristics of secondary thrombotic thrombocytopenic purpura （secondary TTP）and improve diagnostic ability for physician in TTP management.Methods 17 secondary TTP patients admitted to our hospital from 2003 to 2013 were analyzed.Results There were 10 patients（58.82％）with the triad of TTP,including hemolyticanemia,thrombocytopenia and neurologicabnormalities ;8 patients（35.29％）had the classical pentad of TTP.The schistocytes of peripheral blood smears were present in all cases with arrange of 1.5 ％ ～ 16.5％.In the studies,plasmapheresis or plasma infusion in combination with glucocorticiod and immunosuppressor is the main therapeutic method.11 of 17 patients （64.71％）achieved complete remission.Conclusion The diagnosis of secondary TTP is still based on clinical features,and make RECFragments count ＞ 1％ as diagnostic criteria is conducive to early diagnosis and effective treatment.To be active to treat protopathy,and use the therapy of plasmapheresis or plasma infusion combine with glucocorticiod or other immunosuppressor is help survival.

关 键 词：继发性血栓性血小板减少性紫癜 血浆置换 临床特征 

分 类 号：R558[医药卫生—血液循环系统疾病]