血栓性血小板减少性紫癜临床分析

Cases Analysis and Literature Review of Thrombotic Thrombocytopenic Purpura

目的:探讨血栓性血小板减少性紫癜(TTP)的临床特点、诊断要点及治疗方法。方法:分析7例患者的临床特点、血液学特征性改变、影像学特征及实验室检查结果。结果:急性发病,临床表现为贫血、皮肤淤斑、鼻出血、黑便等血液学的异常;头痛、头晕、意识障碍、神经功能缺损或癫痫发作;黄疸、血尿及发热等,实验室检查为溶血性贫血、血小板减少和肾功能异常,神经影像学表现为脑梗死灶或伴出血。在明确诊断后应用激素、血浆置换治疗及对症治疗,7例中2例基本痊愈,2例放弃治疗,3例死亡。结论:对临床急性起病的表现为贫血、出血、头痛、神经功能缺损或癫痫发作的患者,结合血小板减少应高度怀疑TTP,及时进行骨髓像等血液学检查,一旦确诊,及早给予激素、血浆置换治疗及相应对症治疗具有挽救生命的重要意义。

Objective：To investigate clinical features,diagnostic points and treatment methods of thrombotic thrombocytopenic purpura（TTP）.Methods：To perform a retrospective observational review of 7 patients with TTP to analyze their clinical features,hematological characteristics,imageological changes and laboratory results.Results：TTP is an acute illness,its clinical manifestations included anaemia,skin ecchymosis,nose bleed,dark stools in hematological abnormality;headache,dizziness,conscious disturbance,and/or epileptic attack in neurological dysfunctions;jaundice,blood urine and fever.Laboratory examinations consisted of hemolytic anemia,thrombocytopenia and renal dysfunction.Neuroimaging showed cerebral infarction,or accompanied with hemorrhage.After definite diagnosis,dexamethasone or methylprednisolone,plasma exchange or freezed fresh plasma and symptomatic treatment were used to rescue patients,two of them were almost recovery,two gave up treatment due to cost of hospitalization and other three died.Conclusion：It should be highly regarded as TTP if an acute illness shows anaemia,hemorrhage,headache,neurological abnormality or epilepsy,and its laboratory examination demonstrates thrombocytopenia,then hematological detection,brain CT or MRI,liver function and renal function should be examined to identify diagnosis,it is important to take managements as soon as possible to rescue patients if it is definite TTP.