患有二次性血细胞性淋巴细胞瘤的儿童的凝血功能障碍:综合分析
Chaojun Duan1, Qing Liao1, Jiale Gong1
1Department of Laboratory Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
凝血功能障碍显著影响儿科二级血细胞性淋巴细胞瘤 (sHLH). 在sHLH的儿童中,前热血素时间,D-二次体和纤维素原的异常预测了糟糕的结果和分散的血管内凝血 (DIC) 风险.
科学领域:
- 儿科血液学 儿科血液学
- 凝血障碍 凝血障碍 凝血障碍
- 免疫学 免疫学 免疫学
背景情况:
- 凝血功能障碍是儿童二级血细胞淋巴细胞瘤 (sHLH) 发病和预后的一个关键因素.
- 了解这些凝血概况对于改善患者的治疗结果至关重要.
研究的目的:
- 系统地分析儿科sHLH患者的凝血概况.
- 评估这些凝血参数的预后值.
- 建立一个减少儿童sHLH死亡率的基础.
主要方法:
- 在入院时对209名儿科sHLH患者的凝血参数 (PT,INR,APTT,TT,D-二次体,纤维素) 的分析.
- 对病因和预后组的参数进行比较,包括那些有扩散性血管内凝血 (DIC) 的人.
- 统计分析包括LOWESS曲线拟合,LASSO回归,后勤回归和Kaplan-Meier生存分析.
主要成果:
- 儿童sHLH患者在入院时表现出显著的凝血异常,包括升高的PT,INR,APTT,TT和D-二次体.
- 与自身免疫相关的HLH相比,与感染相关的HLH显示出更严重的凝血障碍.
- 升高的PT,INR和D-二次体与死亡率有关,而神经参与和DIC是死亡率的独立预测因素.
结论:
- 凝血功能障碍是儿科sHLH的中心病理特征,特别是在感染相关的病例中.
- 对凝血参数和费里水平的动态监测对于风险评估和早期干预至关重要.
- 管理凝血异常和神经复杂症是改善儿科sHLH结果的关键.
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