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在患有轻度遗传性血小板功能障碍的儿童中,ISTH的出血评估工具和血小板功能分析仪
Dana Alhaj1, Nikola Hagedorn1, Franziska Cuntz1
1Department of Pediatric Hematology and Oncology, Charité University Medicine, Berlin, Germany.
European journal of haematology
|March 29, 2024
概括
血小板功能分析仪 (PFA) 和ISTH-BAT工具不能有效地诊断儿童轻度遗传性血小板功能障碍. 对于排除像VWD这样的严重疾病来说,PFA更好.
科学领域:
- 儿科血液学 儿科血液学
- 诊断医学 诊断医学 诊断医学
- 血液静止和血栓形成
背景情况:
- 轻度遗传性血小板功能障碍 (IPFD) 在儿童中存在诊断挑战.
- 准确的诊断对于适当的管理和预防出血并发症至关重要.
研究的目的:
- 评估血小板功能分析仪 (PFA) 和国际血栓形成和血静止出血评估工具 (ISTH-BAT) 的诊断实用性,用于检测儿科患者的轻度IPFD.
- 将PFA和ISTH-BAT的性能与包括遗传查在内的全面诊断工作进行比较.
主要方法:
- 对97名怀疑患有出血障碍的儿童 (<18岁) 进行前性单中心研究.
- 标准化工作包括ISTH-BAT,PFA (使用腺二酸盐和上腺素盒),血小板聚合,免疫光和下一代测序.
- 患者被分为·维勒布兰德病 (VWD),其他IPFD或没有诊断.
主要成果:
- PFA和ISTH-BAT的组合模型在诊断IPFD方面表现低 (AUC0.56),但在VWD方面表现更高 (AUC0.84).
- 在VWD和Glanzmann血栓塞栓症中,PFA结果延长,而ISTH-BAT得分仅在严重的出血障碍中呈阳性,缺少轻度的IPFD和VWD.
- 在PFA和ISTH-BAT之间观察到较低的相关性 (斯皮尔曼系数0.25-0.39).
结论:
- 无论是ISTH-BAT还是PFA,单独或组合,都不是诊断儿童轻度IPFD的有效工具.
- 在儿童患者中,PFA在排除严重的IPFD和VWD方面表现出实用性,在这个特定的角色中超过ISTH-BAT.
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