摘要
血栓性血小板减少性紫癜(TTP)是临床较为少见的血液系统急症,多系统受累,临床表现呈现多样化、复杂化,缺乏特异性的诊断标准。本文报道了21例TTP患者的临床表现、实验室检查结果、治疗及转归。21例均为获得性TTP,其中19例属特发性,首发症状涉及多个系统,三联症者19例(90.5%),三联症伴肾功能损害者5例(23.8%);10例中9例(90%)血管性血友病因子裂解酶(ADAMTS13)活性降低;14例行血浆置换者11例(78.6%)缓解出院,7例未行血浆置换者只1例存活。急诊遇到Coombs试验阴性的溶血性贫血和血小板减少时,即应考虑TTP的可能,尤其是合并有神经精神症状者,ADAMTS13检测有助于诊断。早期诊断并尽早给予血浆置换治疗,是急诊TTP患者良好预后的保证。
Thrombotic thrombocytopenic purpura（ TTP） is a rare hematological emergency,which involves multiple systems,so clinical manifestations of TTP patients are diverse and complicated. Moreover, there is no specific criteria for the diagnosis of TTP. This paper reported the clinical manifestations,laboratory findings,management and outcomes of 21 patients with TTP. These patients all had acquired TTP,and 19 cases were idiopathic TTP. Initial symptoms involved various systems,with triad found in 19 cases（ 90. 5%） and triad combined with renal function damage found in 5 cases（ 23. 8%）. ADAMTS13 activity was detected in 10 patients, of which 9 cases（ 90%） decreased. Of 14 cases with plasma exchange, 11 cases（ 78. 6%） had remission,but of 7 cases without plasma exchange,only one patient survived. In the case of patients with Coombs test negative hemolytic anemia and thrombocytopenia,the possibility of TTP should be considered,especially those patients with neuropsychiatric symptoms,and the detection of ADAMTS13 is helpful for diagnosis. Early diagnosis and early administration of plasma exchange are the guarantee for good prognosis in patients with TTP.
出处
《中国全科医学》
CAS
CSCD
北大核心
2016年第8期951-953,957,共4页
Chinese General Practice
