在手术和分娩期间, δ-储存池疾病的高出血率
Elleke van Heerwaarde1, Annick de Moor1, Albert Huisman2
1Center for Benign Haematology, Thrombosis and Haemostasis, Van Creveldkliniek, Utrecht University, Utrecht, The Netherlands.
Research and practice in thrombosis and haemostasis
|December 9, 2025
概括
预防性治疗显著减少了接受手术的三角储存池疾病 (δ-SPD) 患者的出血并发症. 早期诊断和预防措施对于管理这种血小板疾病至关重要.
科学领域:
- 血液学 血液学 血液学
- 血小板疾病 血小板疾病
- 血液静止 血液静止 血液静止
背景情况:
- 三角储存池疾病 (δ-SPD) 的特征是血小板中缺乏密集颗粒分泌.
- 这种缺陷导致出血倾向增加,特别是在手术和分娩期间.
研究的目的:
- 评估预防性治疗在诊断为 δ-SPD 的患者减少出血并发症的有效性.
- 分析诊断时间和预防治疗对出血率的影响.
主要方法:
- 对38名在2016年至2024年期间被诊断为 δ-SPD患者的医疗记录的回顾性审查.
- 在侵袭性手术,手术和诊断前后分娩期间分析出血并发症.
- 评估治疗策略,包括预防性血小板输血.
主要成果:
- 整体出血率显著下降,从诊断前的51.5%降至诊断后的16.8% (P < .001).
- 接受预防治疗的患者与没有 (45.8%) (P <.001) 的患者相比,出血率较低 (17.9%).
- 用预防性血小板输血进行剖腹产时,没有出血,而没有预防的阴道分娩10例中有6例经历了分娩后出血.
结论:
- δ-SPD患者经历了相当大的外科手术前和外科周边出血风险.
- 及时诊断和实施预防性治疗策略可以显著缓解d-SPD的出血并发症.
- 预防性血小板输血是有效的预防在剖腹产期间出血在d-SPD患者.
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