血小板最大聚集率和血栓最大振幅在儿童血小板无力症和巨大血小板综合征中的临床应用

Clinical application of maximum aggregation rate and thrombus maximum amplitude in children with Glanzmann thrombasthenia and Bernard-Soulier syndrome

目的探讨不同致聚剂诱导的血小板最大聚集率(MAR)和血栓弹力图(TEG)参数血栓最大振幅(MA)在血小板无力症(GT)和巨大血小板综合征(BSS)中的诊断价值。方法选取2014年7月—2021年6月首都医科大学附属北京儿童医院24例GT患儿(GT组)和13例BSS患儿(BSS组),以20例凝血因子缺乏症患儿作为对照组。采用光学比浊法(LTA)和TEG比较各组血小板聚集功能的差异。结果GT组二磷酸腺苷(ADP)、胶原蛋白(Col)、花生四烯酸(AA)诱导的MAR和MA值均显著低于对照组和BSS组(P<0.01);瑞斯托霉素(Risto)诱导的MAR显著低于对照组(P<0.01);BSS组Risto诱导的MAR显著低于对照组和GT组(P<0.01)。Spearman相关分析结果显示,各组4种致聚剂诱导的MAR与MA值均无相关性(P>0.05)。结论联合多种致聚剂检测MAR可更好地反映伴血小板功能障碍的先天性出血性疾病患儿血小板聚集功能,MAR对GT和BSS的诊断和鉴别更有意义,MA值不适合作为BSS的诊断依据。

Objective To compare the difference between the maximum amplitude(MA)values of thromboelastography(TEG)and maximum aggregation rate(MAR)of different agglomerators in Glanzmann thrombasthenia(GT)and Bernard-Soulier syndrome(BSS).Methods Totally,from July 2014 to June 2021,24 GT and 13 BSS patients were enrolled from Beijing Children's Hospital of Capital Medical University,and another 20 patients with coagulation factor deficiency were enrolled as control group.Light transmittance aggregometry(LTA)and TEG were used to compare the differences in platelet aggregation function among the groups.Results The MA value and MAR induced by adenosine diphosphate(ADP),collagen(Col)and arachidonic acid(AA)of GT group were lower than those of BSS group and control group(P<0.01).Ristocetin(Risto)induced MAR in GT group was lower than that in control group(P<0.01).Risto induced MAR in BSS group was lower than those in GT group and control group(P<0.01).Spearman correlation analysis showed that there was no correlation between MAR and MA values induced by the 4 agglomerators in GT,BSS and control group(P>0.05).Conclusions The combined determination of MAR with multiple agglomerators can reflect the platelet function of children with congenital bleeding disorders better.Therefore,MAR is more sensitive than MA value in diagnosing and distinguishing GT and BSS.MA value can not be a diagnostic marker of BSS.