血小板无力症45例临床分析  

Analysisto the Clinical Characteristics of 45 Glanzmann’s Thrombasthenia Patients

作　　者：许剑辉 李大鹏 田萌苏 任红英 薛艳萍 

机构地区：[1]中国医学科学院北京协和医学院血液病医院血液学研究所,天津300020

出　　处：《血栓与止血学》2013年第3期97-102,共6页Chinese Journal of Thrombosis and Hemostasis

基　　金：国家自然科学基金资助项目(81000196)

摘　　要：目的回顾近年来血小板无力症(glanzmann’s thrombasthenia,GT)临床诊断治疗的现状,探讨进一步精确诊断的必要性。方法分析我中心过去7年间收治的45例确诊和临床疑诊GT患者临床资料、出血积分和血小板聚集试验结果,并和同期收治的另一遗传出血性疾病vWD进行比较。结果临床确诊和疑诊GT的患者分别为34例和11例,其中确诊患者的两个或以上出血表现发生率较疑诊患者明显增多(P<0.05);二磷酸腺苷(ADP)和花生四烯酸(AA)诱导的血小板聚集均较疑诊GT患者、血管性血友病(von Willebrand’s disease,vWD)患者显著低下(P<0.05);疑诊GT患者ADP和胶原(Col)诱导的血小板聚集均较vWD患者显著低下(P<0.01),确诊GT患者Col诱导的血小板聚集较vWD患者也显著低下(P<0.01),而瑞斯托霉素(Risto)诱导的血小板聚集较vWD患者显著增高(P<0.01)。确诊GT的患者中,出血积分和ADP诱导的、Col诱导的、AA诱导的血小板聚集水平间无显著相关性。GT患者的临床出血积分和贫血发生显著相关(P<0.05)。结论临床出血积分可更好地反映GT患者出血状况,临床确诊的GT患者诊断较为可靠,疑诊GT的患者诊断可能存在异质性。Objective To make an overview on the current clinical diagnosis and treatment of glanzmann＇ s thrombasthenia （GT） patients, and to clarify the necessity of precise diagnosis of GT in our center. Methods 45 definitely diagnosed and provisionally diagnosed GT patients＇ cases and laboratory test results were collected and clinical bleeding scores were counted,with comparison to 50 contemporarily diagnosed patients with another hereditary bleeding disease, vWD. Results The definitely diagnosed and provisionally diagnosed GT group in clinic had 34 and 11 patients respectively. The happening rate of 2 or more bleeding manifestations was higher in definitely diagnosed GT patients than in provisionally diagnosed GT patients （P 〈 0.05 ）. The adenosine diphosphate （ADP） or arachidonic acid （AA） induced platelet aggregation rates were lower in definitely diagnosed patients than in provisionally diagnosed GT patients or vWD patients （P 〈 0.05 ）. The provisionally diagnosed GT patients had lower ADP or collagen （Col） induced platelet aggregation rates than vWD patients （P 〈 0.01 ）. Definitely diagnosed GT patients had lower Col induced platelet aggregation rates and higher ristomycin induced platelet aggregation rates. Although no obvious relationship between clinical bleeding score and ADP, Col and AA induced platelet aggregation were found in definitely diagnosed GT patients, the score obviously correlated with anemia in clinically diagnosed GT patients. Conclusion The definitely diagnosed GT patients were much consistent. The provisionally diagnosed GT patients had heterogeneity in clinical manifestation and laboratory test. The bleeding score had a better power in reflecting the clinical manifestations in GT patients.

关 键 词：血小板无力症 血小板聚集实验 临床出血积分 

分 类 号：R558.1[医药卫生—血液循环系统疾病]