罕见的凝血因子缺陷:来自印度南部单一三级护理中心的五年经验
Nivedita Suresh1, Bitty Kurian1, Reshma Jeladharan1
1Department of Pathology, Amrita Institute of Medical Sciences.
概括
罕见的凝血因子缺陷 (RCFD) 是不常见的出血障碍. 最常见的是VII因子缺乏,大多数患者的手术结果都很好,这表明需要对管理指南进行更多研究.
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
- 罕见疾病 罕见疾病
背景情况:
- 罕见的凝血因子缺陷 (RCFD) 是一组多样化的疾病,除了 VIII,IX 和·维勒布兰德因子 (VWF) 之外,还影响了凝血因子.
- 关于它们的地理流行,出血模式和临床行为,人们对它们的理解有限.
- 本研究解决了有关RCFD特征和结果的知识差距.
研究的目的:
- 调查RCFD的频率和临床表现.
- 评估因子缺陷的严重程度和血液产品需求.
- 评估RCFD患者的手术结果.
主要方法:
- 从2018年9月到2023年10月进行了一项回顾性队列研究.
- 数据包括专业凝血实验室诊断的患者的临床特征和出血表型.
- 完整的凝血工作被用于患者的诊断.
主要成果:
- 在1019名被评估的患者中,有93人 (9.1%) 患有RCFD,男性占主导地位 (M:F比率为 2:1).
- 第七因子缺乏是最常见的RCFD (40%),粘膜皮肤出血是最常见的症状.
- 大约一半的RCFD患者有症状,接受手术的患者没有任何不良结果.
结论:
- 第七因子缺乏是最常见的RCFD,并且很大一部分患者仍然没有症状.
- RCFD患者通常在手术和怀孕环境中经历了良好的结果.
- 需要进一步的研究来制定管理指南,特别是来自资源有限的地区.
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