21例血栓性血小板减少性紫癜的临床分析

目的总结血栓性血小板减少性紫癜（TTP）的临床特点,并进行疗效分析。方法回顾性分析21例TTP患者的临床资料和治疗情况。结果 21例患者,中位年龄38岁,＂五联征＂在入院就诊的突出临床表现中分别为13,12,16,1和7例次,结合实验室检查其发生率分别为100%、100%、95%、67%和67%,其中95%患者出现＂三联征＂,57%出现＂五联征＂。16例患者给予血浆置换（PE）或新鲜冰冻血浆输注（PI）治疗,PE组8例均治愈,PI组4例治愈、2例复发、2例病情恶化昏迷死亡,两组治愈率分别为100%和50%,差异有统计学意义（P=0.038）。结论 TTP患者以＂三联征＂为突出临床表现,PE疗效显著优于PI,神志清醒程度降低患者应尽早PE治疗。

Clinical Analysis of 21 Patients with Thrombotic Thrombocytopenic Purpura

Objective To summarize the clinical features and treatment of thrombotic thrombocytopenic purpura（TTP）.Methods The clinical and laboratory data of 21 cases with TTP were reviewed retrospectively.Results Median age of the 21 patients was 38 years.As the initial clinical presentation,the classic pentad of thrombocytopenia,microangiopathic hemolytic anemia（MAHA）,fluctuating neurological signs,renal dysfunction,and fever was 13 episodes,12 episodes,16 episodes,1 episode and 7 episodes.The pentad of clinical and/or laboratory abnormalities presented at diagnosis in 100%,100%,95%,67% and 67% of cases,respectively.Ninty-five percent of TTP patients presented with the triad of thrombocytopenia,MAHA,and neurologic dysfunction,and only 57% with the classic pentad signs.16 patients after diagnosis received either plasma exchange（PE） or plasma infusion（PI）.With follow-up（median,42 months;range 9 to 118 months）,eight patients in PE group were all cured.Of the 8 patients treated with PI,four patients were cured;two relapsed;two deteriorated and died.The efficacy of PE had significantly better outcome than that of PI（cure rate：100% versus 50%,P=0.038）.Conclusion The most prominent clinical abnormalities of TTP at presentation are the triad of thrombocytopenia,MAHA and fluctuating neurological signs.PE is more efficacious than PI.If reduced level of consciousness has been identified,PE should be instituted as soon as practicable.