以神经系统症状为首发表现的血栓性血小板减少性紫癜的临床分析.docxVIP

以神经系统症状为首发表现的血栓性血小板减少性紫瘢的临床III III 分析罗琳聂彦博陈飞 刘明辉左学兰(通讯作者)(武汉大学中南医院，湖北武汉430071) 摘要 目的：探讨以神经系统症状为首发表现的血栓性血小板减少性紫瘢(TTP)患者的临床特 征及诊治策略，提高对该病的认识。 方法：回顾性分析2011年1月至2016年11月在我院就诊的以神经系统症状为突出表现的12例 TTP患者的一般资料、临床表现、实验室及影像学检查资料、治疗方法以及转归。 结果：神经系统表现多样，主要有头痛、头晕、认知障碍、昏迷等，仅3例头部CT示多发腔 隙性脑梗塞，余无异常；其余症状有紫瘢出血、溶血、发热、肾功能损害等。采用血浆置换、 糖皮质激素等治疗，7例有效。 结论：以神经系统为首发表现的TTP易发生误诊，我们需提高队该病的认识。血浆置换加糖 皮质激素为标准治疗，难治性病例可应用利妥昔单抗或其他免疫抑制剂治疗。 关键词：血栓性血小板减少性紫瘢；神经系统表现；血浆置换；糖皮质激素；利妥昔单抗 Abstract Objective To investigate the clinical characteristics, diagnosis and treatment of thrombotic thrombocytopenic purpura (TTP) patients with neurological symptoms as the first manifestation, and to improve the understanding of the disease. Methods The general data, clinical manifestations, laboratory and imaging data, treatment methods and outcome of 12 TTP patients with neurological symptoms were analyzed retrospectively from January 2011 to November 2016 in our hospital. Results The nervous system showed a variety of performances, mainly include headache, dizziness, cognitive disorders, coma and so on. Only 3 cases of head CT showed multiple lacunar infarction, there were no other obvious abnormalities; the other symptoms like bleeding, hemolysis, fever, renal dysfunction. Using the treatment of plasma exchange and glucocorticoid, 7 cases were effective. Conclusion Thrombotic thrombocytopenic purpura (TTP) patients with neurological symptoms as the first manifestation were prone to misdiagnosis, We need to raise the awareness of TTP. Plasma exchange combined with glucocorticoid were the standard treatment, while the recurrence or refractory cases should be applied to the rituximab as soon as possible; patients who occurred rituximab resistance need to consider the application of other immunosuppressive agents such as vincristine or new drug treatment. Key words thrombotic thrombocytopenic purpura; nervous system performance; plasma exchange; glucocorticoid; rituximab 血栓性血小板减少性紫瘢(Thrombotic Thrombocytopenic Purpura, TTP)是一种严重