Utility of the ISTH bleeding assessment tool in predicting platelet defects in participants with suspected inherited platelet function disorders
Utility of the ISTH bleeding assessment tool in predicting platelet defects in participants with suspected inherited platelet function disorders
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DOI：
10.1111/jth.12332
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发表时间：
2013-09-01
影响因子：
10.4
通讯作者：
Watson, S. P.
中科院分区：
文献类型：
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作者：
Lowe, G. C.;Lordkipanidze, M.;Watson, S. P.
ISTH出血评估工具ISTH-BAT用于记录出血症状并辅助诊断可能存在出血性疾病的患者。目的探讨ISTH-BAT在预测疑似遗传性血小板功能障碍患者血小板活化功能缺陷中的实用性。方法对有大量出血和疑似遗传性血小板功能障碍的临床证据的参与者和健康志愿者进行基因分型，血小板表型分析研究（GAPP; ISRCTN 77951167）。ISTH-BAT问卷调查由一个训练有素的调查员lumiaggregometry.ResultsOne百名参与者被列入（79名疑似遗传性血小板功能障碍，和21名健康志愿者）。疑似遗传性血小板功能障碍受试者的ISTH-BAT评分（中位数12;四分位距[IQR] 8-16）显著高于健康志愿者（中位数0; IQR 0-0）。疑似遗传性血小板功能障碍伴血小板缺陷的受试者（中位数11; IQR 8-16）与血小板功能正常的受试者（中位数12; IQR 8-14）之间无差异（P > 0.05）。在血小板功能检测中，ISTH-BAT评分与明显的血小板缺陷无关（受试者工作特征曲线下面积= 0.501 [95%置信区间0.372-0.630，P = 0.98]和比值比1.01 [95%置信区间0.93-1.09，结论ISTH-BAT是记录终生出血史的有力工具。然而，在疑似遗传性血小板功能障碍的患者中，获得的评分不能预测光聚集测定法上血小板缺陷的存在。
SummaryBackgroundThe ISTH bleeding assessment tool (ISTH-BAT) was developed to record bleeding symptoms and to aid diagnosis in patients with a possible bleeding disorder.ObjectivesTo investigate the utility of the ISTH-BAT in predicting functional defects in platelet activation in participants with suspected inherited platelet function disorders.Patients/MethodsParticipants with clinical evidence of excessive bleeding and suspected inherited platelet function disorders and healthy volunteers were recruited to the Genotyping and Phenotyping of Platelets study (GAPP; ISRCTN 77951167). The ISTH-BAT questionnaire was applied by a trained investigator prior to lumiaggregometry.ResultsOne hundred participants were included (79 with suspected inherited platelet function disorders, and 21 healthy volunteers). The ISTH-BAT score in participants with suspected inherited platelet function disorders (median 12; interquartile range [IQR] 8-16) was significantly higher than in healthy volunteers (median 0; IQR 0-0). There was no difference between participants with suspected inherited platelet function disorders with a platelet defect detected by lumiaggregometry (median 11; IQR 8-16) and those with normal platelet function (median 12; IQR 8-14) (P > 0.05). The ISTH-BAT score was not associated with a demonstrable platelet defect on platelet function testing (area under the receiver operating characteristic curve = 0.501 [95% confidence interval 0.372-0.630, P = 0.98] and odds ratio 1.01 [95% confidence interval 0.93-1.09, P = 0.91]).ConclusionsThe ISTH-BAT is a powerful tool for documenting lifelong bleeding history. However, the score obtained is not predictive of the presence of a platelet defect on lumiaggregometry in patients with suspected inherited platelet function disorders.