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目的 分析总结血小板无力症(GT)患儿的临床特征和实验室资料。方法 对 1990~2002年中国医学科学院中国协和医科大学血液学研究所血液病医院收治的 45例GT患儿的临床表现、实验室资料、治疗措施进行回顾分析。结果 45例血小板无力症患儿的血小板对多种诱导聚集剂(ADP、AA、COLADR等 )反应低下或(和)缺如;瑞斯托霉素诱导的血小板聚集正常。结论 多种诱导聚集剂诱导的血小板聚集试验是诊断GT的关键。
Objective To analyze the clinical features and laboratory data of children with thrombocytopenia (GT). Methods The clinical manifestations, laboratory data and treatment measures of 45 GT patients admitted to Hematology Hospital of Chinese Academy of Medical Sciences and Peking Union Medical College from 1990 to 2002 were analyzed retrospectively. Results The platelets in 45 children with PTT were poorly responsive and / or absent to various inducers (ADP, AA, COLADR, etc.); Ristocetin induced platelet aggregation was normal. Conclusion A variety of induced aggregator-induced platelet aggregation test is the key to diagnosis of GT.