识别沉默缺陷:严重的XI因子缺陷在一个无症状的病人与孤立的延长激活的部分血栓形成时间-A病例报告
Awni Alshurafa1, Mahmoud Draidi2, Feryal Ibrahim3
1Hematology Department, Hamad Medical Corporation, Doha, Qatar.
SAGE open medical case reports
|December 29, 2025
概括
这个案例研究突出了一个无症状的男性,偶尔发现了严重的XI因子缺乏症. 一个个性化的术后策略确保了安全的外科手术结果,强调对罕见出血障碍的个性化护理.
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
背景情况:
- 延长的激活部分血栓形成时间 (aPTT) 表明凝血因子问题.
- 十一因子缺乏症是一种罕见的自体逆流性出血障碍,具有不可预测的出血风险.
- 评估出血障碍需要仔细评估,特别是当aPTT延长时.
研究的目的:
- 报告无症状患者偶然发现的严重XI因子缺乏症病例.
- 描述一个患有XI因子缺乏症的患者接受手术的术后管理策略.
- 强调因子XI缺乏症患者个性化管理的重要性.
主要方法:
- 在手术前评估期间,在一名24岁的男性中偶然发现了延长的aPTT.
- 混合研究,以调查长期aPTT的原因.
- 因子检测证实了严重的XI因子缺乏.
- 实施一个量身定制的外科手术期间计划,包括新鲜冷血和tranexamic acid.
主要成果:
- 这名患者被诊断出患有严重的XI因子缺乏症,尽管他没有症状,以前没有出血史.
- 混合研究显示完全纠正,表明因子缺乏而不是抑制剂.
- 实施的术后计划,包括新鲜冷血和松酸,是有效的.
- 患者接受了血栓形成手术,手术和术后过程没有任何事件.
结论:
- 严重的XI因子缺乏症可能是无症状的,并偶然发现.
- 对于XI因子缺乏的患者来说,个性化的术后管理至关重要,即使出血表型不确定.
- 预防措施和随时可用的救援疗法有助于在罕见的出血疾病中取得成功的外科治疗结果.
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