CAJ | 학술논문

目的：探讨血栓性血小板减少性紫癜（thrombotic thrombocytopenic purpura，TTP）的临床特征、治疗及预后。方法将南京医科大学第一附属医院2006年4月至2013年1月诊断的TTP患者21例，分成有效组和死亡组，进行回顾性分析。其中男性6例，女性15例，年龄（46.81±12.17）岁。纳入标准：（1）无明显原因的血小板减少（＜100×109 L-1）；（2）溶血性贫血，Coombs’试验阴性，外周血涂片出现碎裂红细胞；（3）符合上述标准的TTP患者，其临床表现及病程均被纳入本研究。排除标准：（1）＜24 h出院或死亡；（2）在外院已接受血浆置换治疗；（3）病历资料不完整；（4）无法随访；（5）排除其他血栓性微血管病。应用统计分析软件SPSS 20.0对患者一般情况、病因、临床特征、治疗和预后进行分析。符合正态分布的计量资料用（x ± s）表示，组间比较使用t检验；分类资料以频数表示，使用Fisher确切概率法进行比较。P＜0.05为差异具有统计学意义。结果21例患者均有血小板减少及微血管病性溶血性贫血表现，12例（57.2％）出现典型TTP五联征（发热、血小板减少、微血管病性溶血性贫血、神经系统症状和肾脏损害），2例（9.5％）仅表现为三联征（血小板减少、微血管病性溶血性贫血、神经系统症状），7例（33.3％）出现四种临床表现（三联征加上发热或肾脏损害）；本组接受血浆置换治疗者7例，有效率71.4％。结论 TTP病情进展迅速，血浆置换是治疗TTP的首选方法，病因治疗有助于控制病情。
목적：탐토혈전성혈소판감소성자전（thrombotic thrombocytopenic purpura，TTP）적림상특정、치료급예후。방법장남경의과대학제일부속의원2006년4월지2013년1월진단적TTP환자21례，분성유효조화사망조，진행회고성분석。기중남성6례，녀성15례，년령（46.81±12.17）세。납입표준：（1）무명현원인적혈소판감소（＜100×109 L-1）；（2）용혈성빈혈，Coombs’시험음성，외주혈도편출현쇄렬홍세포；（3）부합상술표준적TTP환자，기림상표현급병정균피납입본연구。배제표준：（1）＜24 h출원혹사망；（2）재외원이접수혈장치환치료；（3）병력자료불완정；（4）무법수방；（5）배제기타혈전성미혈관병。응용통계분석연건SPSS 20.0대환자일반정황、병인、림상특정、치료화예후진행분석。부합정태분포적계량자료용（x ± s）표시，조간비교사용t검험；분류자료이빈수표시，사용Fisher학절개솔법진행비교。P＜0.05위차이구유통계학의의。결과21례환자균유혈소판감소급미혈관병성용혈성빈혈표현，12례（57.2％）출현전형TTP오련정（발열、혈소판감소、미혈관병성용혈성빈혈、신경계통증상화신장손해），2례（9.5％）부표현위삼련정（혈소판감소、미혈관병성용혈성빈혈、신경계통증상），7례（33.3％）출현사충림상표현（삼련정가상발열혹신장손해）；본조접수혈장치환치료자7례，유효솔71.4％。결론 TTP병정진전신속，혈장치환시치료TTP적수선방법，병인치료유조우공제병정。
Objective To investigate clinical features, outcomes and laboratory findings of thrombotic thrombocytopenic purpura (TTP).Methods Patients with TTP admitted between April 2006 and January 2013 were identified by a retrospective review of records.Totally 21 patients were available,15 females and 6 males,with a median age of 46 years (ranged 18-66).The diagnostic criteria were defined by:(1)thrombocytopenia (<100 ×9 L-1)without other identifiable causes;(2)a negative Coombs'test and hemolytic anemia with schistocytes on the peripheral blood smear;and only those patients meeting the criteria for TTP,both on clinical presentation and their clinical course,were included in this study. Exclusion criteria were:(1)patients discharged or dead within 24 hours after admission;(2)patients treated with plasma exchange therapy in other hospitals;(3)medical data were incomplete;(4)cannot be followed up;and (5 )other causes of thrombotic microangiopathies.General condition of patients,etiology, clinical features,treatment and prognosis were analyzed by using the SPSS 20.0 software.P value of <0.05 was considered as significant.Results Hemolytic anemia and thrombocytopenia appeared in all of the patients. Twelve patients (57.2%) had the classical pentad manifestations of TTP (fever, thrombocytopenia,microangiopathic hemolytic anemia,symptoms of nervous system,renal injury),seven patients (33.3%)had tetrad of TTP clinical manifestations (thrombocytopenia,microangiopathic hemolytic anemia,symptoms of nervous system,fever or renal injury),and only two patients showed the triad manifestations of TTP (thrombocytopenia, microangiopathic hemolytic anemia, symptoms of nervous system).In our studies,seven patients accepted plasmapheresis,and five of them (71.4%)achieved remission.Conclusions TTP progresses quickly.Plasmapheresis is still the treatment of choice for TTP patients.Etiological treatment can help to control the conditions of patients with TTP.