以神经系统症状为首发的血栓性血小板减少性紫癜26例分析

Neurological manifestations as initial presentations of thrombotic thrombocytopenic purpura:clinical analysis of 26 cases.

目的分析以神经系统症状为首发的血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura,TTP)的临床特点,以提高TTP的早期诊断率。方法收集2002～2010年国内以神经系统症状为首发的TTP患者26例,其中5例为首都医科大学宣武医院神经内科和血液科住院患者,21例来源于7篇文献报道,对临床特点进行分析;根据是否进行血浆置换治疗进行预后分析。结果首发症状表现为神经系统症状的TTP,早期分别被诊断为病毒性脑炎、脑血管病和流行性出血热;疾病早期大多数患者即有不同程度血小板减少,并随着疾病发展进行性下降;头部影像学检查可表现为正常、脑梗死、脑出血和脑白质病变。结论不典型脑炎和脑血管病患者,尤其伴有血液系统异常时,应想到TTP的可能,及早进行外周血涂片并监测血常规和肾功能,早期明确诊断,进行血浆置换治疗。

Objective To analyze thrombotic thrombocytopenie purpnra （TIP） presenting neurological manifestations as initial symptoms. Methods Clinical data of 26 patients from 2002 to 2010 were analyzed including, 5 cases from departments of neurology and hematology of Xuanwu hospital of capital medical university and 21 cases from literature. Results Patients were usually diagnosed with having Encephalitis, Cerebral Vascular Disease and Epidemic Hemorrhagic Fever during early stage. Platelet reduction appeared in early stage and gradually deteriorated over time. Brain imagings of patients could be normal, cerebral infarction, cerebral hemorrhage or abnormal white matter. Compared with non plasma exchange group, the mortality rate in plasma exchange group was reduced apparently. Conclusions Patients with atypical encephalitis and cerebral vascular disease with platelet may be possible TTP. If TTP is confirmed, the first-line treatment of plasma exchange should be initiated to save the patient＇s life.