CAJ | 학술논문

目的：总结分析血栓性血小板减少性紫癜患者的临床表现，实验室特点及治疗，以提高对血栓性血小板减少性紫癜的诊断及治疗水平。方法回顾性分析我院2008年1月~2013年12月诊断的12例血栓性血小板减少性紫癜患者的临床表现、实验室检查及治疗方法。结果①12例患者表现为五联征(微血管病性溶血性贫血、血小板减少、神经精神症状、发热、肾脏损害)者8例，三联征(微血管病性溶血性贫血、血小板减少、神经精神症状)者11例。②所有患者乳酸脱氢酶水平均增高，外周血破碎红细胞比例2~15%。③原发病：病因淋巴瘤1例，抗磷脂抗体综合征LPS 1例，系统性红斑狼疮SLE 1例，免疫因素5例，感染2例，原因不明2例。④治疗以血浆置换、血浆输注、利妥昔单抗、糖皮质激素为主，治疗有效率82.7%。结论血栓性血小板减少性紫癜的早期诊断仍然依赖临床表现，在进行血浆置换、利妥昔单抗等抢先治疗的同时完善相关检查进行排除性诊断。
목적：총결분석혈전성혈소판감소성자전환자적림상표현，실험실특점급치료，이제고대혈전성혈소판감소성자전적진단급치료수평。방법회고성분석아원2008년1월~2013년12월진단적12례혈전성혈소판감소성자전환자적림상표현、실험실검사급치료방법。결과①12례환자표현위오련정(미혈관병성용혈성빈혈、혈소판감소、신경정신증상、발열、신장손해)자8례，삼련정(미혈관병성용혈성빈혈、혈소판감소、신경정신증상)자11례。②소유환자유산탈경매수평균증고，외주혈파쇄홍세포비례2~15%。③원발병：병인림파류1례，항린지항체종합정LPS 1례，계통성홍반랑창SLE 1례，면역인소5례，감염2례，원인불명2례。④치료이혈장치환、혈장수주、리타석단항、당피질격소위주，치료유효솔82.7%。결론혈전성혈소판감소성자전적조기진단잉연의뢰림상표현，재진행혈장치환、리타석단항등창선치료적동시완선상관검사진행배제성진단。
Objective To investigate clinical features,outcome and laboratory characteristics of thrombotic thrombocytopenic purpura (TTP) and improve diagnostic ability for physician in TTP management. Methods Totally 12 diagnosed TTP from 2008.1 to 2013.12 were analyzed clinical features, laboratory characteristics and treatment. Results ①There were 8 patients with the pentad of TTP,including hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever and kidney injury. 11 patients had triad of TTP. ②Al the patients had high level of lactate dehydrogenase, and the schistocytes of peripheral blood smears were present in al cases with a arrange of 2%~15%. The primary disease: 1 cases of lymphoma, antiphospholipid antibody syndrome and 1 cases of LPS, 1 cases of systemic lupus erythematosus SLE, immune factors in 5 cases, infection in 2 cases, 2 cases with unknown causes.④The treatment with plasmapheresis, plasma infusion, rituximab, glucocorticoid, the ef ective rate of treatment 82.7%.Conclusion The early of diagnosis of TTP is stil based on clinical feature, dif erential diagnosis should be completed at the time that of preemption of treatment with plasma exchange and rituximab.