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血友病A的表型严重程度的评估:使用旋转血栓形成显微镜 (ROTEM) 和APTT-凝块波形分析

Deepika Gupta¹, Vandana Arya¹, Jasmita Dass²

¹Department of Hematology, Sir Gangaram Hospital, Old Rajinder Nagar, New Delhi, 110060, India.

|May 14, 2024

概括

旋转性血小板造影 (ROTEM) 有效地区分了有抑制剂和没有抑制剂的严重血友病A患者. 激活的部分血栓形成蛋白时间凝块波形分析 (APTT-CWA) 无法区分这些组.

科学领域:

血液学血液学血液学
凝血障碍凝血障碍凝血障碍
血栓形成研究研究

背景情况:

血友病A (HA) 是一种因因子VIII (FVIII) 水平降低而导致的X链接遗传出血疾病.
一部分严重HA (SHA) 患者表现出不典型的出血模式.
现型严重性评估对于管理HA至关重要.

研究的目的:

评估旋转性血栓电平测量 (ROTEM) 和激活的部分血栓塑时间凝结波形分析 (APTT-CWA),以评估血友病A的表型严重程度.
为了区分使用抑制剂和不使用这些方法的严重血友病A患者.

主要方法:

招募了66名患有血友病A型的患者,并根据表型分类.
使用ROTEM (INTEM模式) 和APTT-CWA分析了全血样本.
临床表型的分配遵循了既定的文献标准.

主要成果:

在四个表型组中,ROTEM和APTT-CWA显示了统计学上显著的差异.
具有抑制剂的SHA患者的ROTEM参数 (MCF,MA30,MAXV,阿尔法角度) 最低,使其与没有抑制剂的SHA患者区别开来.
APTT-CWA参数没有区分有或没有抑制剂的SHA患者.

结论:

通过多种参数的组合,ROTEM可以区分具有抑制剂的SHA患者和没有抑制剂的SHA患者,具有高灵敏度和特异性.
APTT-CWA不适合在A型血友病中区分这些特定的患者亚组.

关键词:

在APTT-CWA中.贝塞斯达测试试验贝塞斯达测试血液失常症血液失常症血友病A型血友病A型血友病现象型严重程度现象型严重程度罗特姆公司 (ROTEM)

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