血栓性血小板减少性紫癜37例临床分析  

Thrombotic thrombocytopenic purpura:Clinical analysis of 37 cases

作　　者：邓明扬[1] 张广森[1] 张扬[2] 杨景柯[1] 戴崇文[1] 徐运孝[1] 郑文莉[1] 彭宏凌[1] 申健凯[1] 

机构地区：[1]中南大学湘雅二医院血液科,湖南长沙410011 [2]中南大学湘雅二医院肿瘤科,湖南长沙410011

出　　处：《现代生物医学进展》2010年第2期267-270,共4页Progress in Modern Biomedicine

摘　　要：目的:总结分析血栓性血小板减少性紫癜(TTP)患者临床表现,实验室特点及治疗反应,以提高TTP诊断水平和治疗效果。方法:对我院1998年1月～2009年4月期间诊断为TTP的37例患者进行临床特征和治疗转归分析,对所有病例的外周血涂片进行破碎红细胞计数,计数5000个红细胞中所含裂红细胞进行定量。结果:37例患者仅7例(18.9%)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);30例TTP患者(81.1%)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)。本组病例中主要的致病因素为自身免疫性疾患,特发性TTP占56.76%。本组病例外周血涂片100%存在破碎红细胞,均值为4.4%,范围为0.3%～13.4%。本组病例的治疗以血浆置换和输注血浆为主,治疗有效率88.46%;接受血浆输注治疗的患者缓解率仅为18%,而死亡率为82%。结论:TTP诊断仍然依赖临床表现特点,破碎红细胞计数是非常有价值的诊断指标。血浆置换联用血浆输注仍是治疗TTP的首先方法。Objective：To investigate clinical features,outcome and laboratory characteristics of thrombotic thrombocytopenic purpura（TTP） and improve diagnostic ability for physician in TTP management. Methods：Thirty-seven TTP patients admitted to our hospital from 1998 to 2009 were analyzed. The number of schistocytes per 5,000 red cells was counted at 1000-power magnification. The results were expressed as number of schistocytes per 100 red cells. Results：There were 30 patients（81.1%） with the triad of TTP,including hemolytic anemia,thrombocytopenia and neurologic abnormalities;7（18.9%） had the classical pentad of TTP. Major etiologic factors were acquired autoimmunological abnormalities（35.14%） or idiopathic TTP（56.76%） . The schistocytes of peripheral blood smears were present in all cases with a mean of 4.4% and a range of 0.3%-13.4%. In the studies,plasmapheresis in combination with plasma infusion is the main therapeutic method. 23of 26 patients（88.46%） accepting plasmapheresis achieved complete remission;those patients who only underwent plasma infusion had low remission rate（18%） and high mortality（9/11;82%） . 4 patients with packed RBC infusion manifested transient exacerbation of neurologic or psychiatric symptoms. Conclusions：In conclusion,the diagnosis of TTP is still based on clinical features including evidence of microangiopathic haemolysis and an increased schistocytes count is more valuable and simpler method for the diagnosis of TTP. Plasmapheresis is still the optimal treatment method for TTP patients.

关 键 词：血栓性血小板减少性紫癜(TTP) 血浆置换 临床特征 

分 类 号：R733[医药卫生—肿瘤]