成年开始的维生素K依赖的凝血因子缺乏,病因不明
Yui Araki1, Miyuki Ookura2, Naoko Ida2
1Department Hematology and Oncology, Fukui Prefectural Hospital, Japan.
Internal medicine (Tokyo, Japan)
|September 10, 2025
概括
成年人很少因为维生素K依赖的凝血因子缺乏 (VKCFD) 而出血. 这项研究确定了两名日本成年人获得VKCFD的原因不明,这表明一种新的疾病实体.
科学领域:
- 血液学 血液学 血液学
- 内部医学 内部医学
- 临床研究 临床研究
背景情况:
- 维生素K依赖的凝血因子缺乏症 (VKCFD) 通常在婴儿或幼儿时期出现.
- 在成年人中获得的VKCFD并不常见,其病因通常是可识别的 (例如,肝病,华法林使用).
研究的目的:
- 描述两例未知病因的成人发病VKCFD病例.
- 提出一种新的疾病实体,用于异常获得的VKCFD.
主要方法:
- 两名日本成年患者的病例报告显示出出血症状.
- 综合实验室评估,包括凝血因子水平 (因子VII,IX,X,II),蛋白C,蛋白S和PIVKA-II.
- 排除先天性VKCFD和其他已知的获得VKCFD的原因.
主要成果:
- 这两名患者均表现出多种维生素K依赖因子 (FVII,FIX,FX,FII) 和抗凝蛋白 (蛋白C,蛋白S) 的水平显著降低.
- 在这两种情况下都观察到由维生素K缺失/对手II (PIVKA-II) 诱导的蛋白质水平升高.
- 遗传性VKCFD被排除在外,尽管进行了彻底的调查,但没有发现可识别的维生素K缺乏的原因.
结论:
- 异常获得的VKCFD是成年人潜在的新疾病实体.
- 这种情况的特点是缺乏多种维生素K依赖因素和蛋白质,没有明确的潜在原因.
- 需要进一步的研究来了解这种新提议的实体的病理生理学和患病率.
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