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

Clinical analysis of thrombotic thrombocytopenic purpura with neurological symptoms as the first manifestation

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癜(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示多发腔隙性脑梗死,余无异常;其余症状有紫癜出血、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,我们需提高对该病的认识。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。

Objective：To investigate the clinical characteristics,diagnosis and treatment of thrombotic thrombocytopenic purpura（TTP） 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 including headache,dizziness,cognitive disorders,coma and so on.Only 3 cases of head CT showed multiple lacunar infarction,and there were no other obvious abnormalities.The other symptoms included bleeding,hemolysis,fever,renal dysfunction.Using the treatment of plasma exchange and glucocorticoid,7 cases were effective.Conclusion：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 are the standard treatment,while the recurrence or refractory cases should be given rituximab or other immunosuppressive agents.