CAJ | 학술논문

目的：探讨血栓性血小板减少性紫癜（TTP）患者的临床特征及治疗反应，提高对TTP的诊断及治疗水平。方法：回顾性分析29例患者的临床表现、实验室检查结果、诊治及疗效。结果：29例TTP患者，平均年龄43岁，62%（18/29）为女性。8例（27.6%）临床出现典型五联征（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）；23（79.3%）例TTP患者表现三联征（血小板减少、微血管病性溶血性贫血、神经系统症状）。所有的患者外周血涂片可见破碎红细胞，均值为8.9%（2%-20%）。本组病例以血浆置换（PE）和血浆输注（PI）治疗为主，18例患者予以PE治疗，中位置换次数为4.5次（1-10次），9例患者予以PI治疗，PE组和PI组有效率分别为72.2%和22.2%，差异有统计学意义（P=0.0369）。结论：TTP女性多见，“三联征”为其突出临床表现，血清乳酸脱氢酶及外周血破碎红细胞计数升高对诊断具有重要参考价值。PE疗效显著优于PI ，为治疗TTP的首选方法。
목적：탐토혈전성혈소판감소성자전（TTP）환자적림상특정급치료반응，제고대TTP적진단급치료수평。방법：회고성분석29례환자적림상표현、실험실검사결과、진치급료효。결과：29례TTP환자，평균년령43세，62%（18/29）위녀성。8례（27.6%）림상출현전형오련정（발열、혈소판감소、미혈관병성용혈성빈혈、신경계통증상화신장손해）；23（79.3%）례TTP환자표현삼련정（혈소판감소、미혈관병성용혈성빈혈、신경계통증상）。소유적환자외주혈도편가견파쇄홍세포，균치위8.9%（2%-20%）。본조병례이혈장치환（PE）화혈장수주（PI）치료위주，18례환자여이PE치료，중위치환차수위4.5차（1-10차），9례환자여이PI치료，PE조화PI조유효솔분별위72.2%화22.2%，차이유통계학의의（P=0.0369）。결론：TTP녀성다견，“삼련정”위기돌출림상표현，혈청유산탈경매급외주혈파쇄홍세포계수승고대진단구유중요삼고개치。PE료효현저우우PI ，위치료TTP적수선방법。
Objective To investigate clinical features,out come and laboratory characteristics of thrombotic thrombocytopenic purpura (TTP) and improve diagnostic ability for physician in TTP management. Methods The clinical,laboratory data and treatment of 29 cases with TTP were reviewed retrospectively. Results sixty-two percent of the patients were females.The average age of the pa-tients was 43 years(range:12-66).There were 8 patients(27.6%)with the triad of TTP,including hemolytic anemia,thrombocytopenia and neurologic abnormalities;23(79.3%)had the classical pental of TTP.The schistocytes of peripheral blood smears were present in 29 cases with a median of 8.9%.27 patients after diagnosis received either plasmaexchange(PE) or plasma infusion(PI). The efficacy of PE had significantly better than that of PI( cure rate:72.2%versus22.2%, P=0. 0369). Conclusion TTP is common in female,the most prominent clinical abnormalities of TTP at presentation are the traid of hemolytic anemia,thrombocytopenia and neurologic abnormali-ties,and The increased serum lactate dehydrogenises and schistocytes counts are of great significance for diagnosis.Plasmapheresis is still the optimal treatment method for patients of TTP.