中国全科医学Issue(33)：4119-4121,4122,4.DOI:10.3969/j.issn.1007-9572.2015.33.021
获得性血栓性血小板减少性紫癜16例临床分析
Acquired Thrombotic Thrombocytopenic Purpura:Clinical Analysis of 16 Cases
潘玉夏 1杨琳 1田金满 1尚银涛 1王兴哲 1王茜 1苏金明 2罗建民1
作者信息
- 1. 050000 河北省石家庄市，河北医科大学第二医院血液内科，河北省血液病重点实验室
- 2. 河北省唐山市乐亭县医院呼吸科
- 折叠
摘要
Abstract
Objective To investigate the clinical manifestations,laboratory characteristics,treatment strategies and outcome of patients with acquired thrombotic thrombocytopenic purpura(TTP). Methods A total of 16 acquired TTP patients admitted to the Second Hospital of Hebei Medical University from January 2013 to December 2014 were enrolled in the study. A retrospective analysis of clinical manifestations,laboratory examination data,ADAMTS 13 activity,inhibitor test results, treatment method and efficacy was conducted. Results Five(31. 3% )patients had typical " Reynolds' pentad" of the 16 patients,all the patients had different degrees of microangiopathic hemolytic anemia and thrombocytopenia. 14 patients received peripheral blood smear examination,and broken red blood cells were found in 10 patients, with 6 > 1% . The lactate dehydrogenase(LDH)of 15 patients was increased,and the indirect bilirubin(IBIL)of 16 patients was increased. ADAMTS 13 activity reduced in all patients,with 15 patient less than 10% ;the results of ADAMTS 13 inhibitor test were all positive. Among the 16 patients,2 patients died before treatment,1 patient quitted treatment,the rest 13 patients were administrated with PE combined with corticosteroid,among which 2 patients died within 24 hours after treatment began and 11 patients saw the treatment effective. Conclusion The diagnosis of acquired TTP is based on the presenting clinical features: thrombocytopenia, microangiopathic hemolytic anemia,neurologic abnormalities,renal failure and fever. A severe decrease of ADAMTS 13 activity with the presence of an inhibitor confirms the diagnosis of acquired TTP. Early administration of PE combined with immunosuppressors is the essential element for the effective emergency treatment of acquired TTP.关键词
紫癜,血栓性血小板减少性/血管性血友病因子裂解蛋白酶/血浆置换Key words
Purpura,thrombotic thrombocytopenic/ADAMTS 13/Plasma exchange分类
医药卫生引用本文复制引用
潘玉夏,杨琳,田金满,尚银涛,王兴哲,王茜,苏金明,罗建民..获得性血栓性血小板减少性紫癜16例临床分析[J].中国全科医学,2015,(33):4119-4121,4122,4.
