获得的第八因子 (FVIII) 缺乏症:一种异常出血病例
Ahmad Khalil1, Dina Foudeh2, Ismael Nassar3
1Department of Oncology, Islamic Hospital, Amman, JOR.
Cureus
|November 7, 2025
概括
获得的血友病A,一种罕见的出血障碍,在一个患有自发血瘤的妇女身上被诊断出. 及时诊断和免疫抑制疗法导致了快速恢复和抑制剂消除.
科学领域:
- 血液学 血液学 血液学
- 免疫学 免疫学 免疫学
背景情况:
- 获得性血友病A (AHA) 是一种罕见的自身免疫性疾病,其特点是对凝血因子VIII (FVIII) 产生抑制剂.
- AHA通常表现为自发,严重出血,特别是在老年人或产后妇女中,但也可能出现不典型的表现.
研究的目的:
- 报告一个年轻女性获得的血友病A病例,具有非典型的自发血瘤呈现,没有粘膜皮肤出血.
- 突出诊断挑战,并强调迅速识别和有效的治疗策略的重要性.
主要方法:
- 一名34岁的女性没有先前出血史,出现了自发性血瘤.
- 诊断工作包括实验室测试 (aPTT,FVIII活性,抑制剂标位) 和成像 (超声波).
- 治疗包括用甲基prednisolone,环胺,prednisolone和阿扎西奥普林进行免疫抑制疗法.
主要成果:
- 实验室检测结果显示,激活部分血栓形成时间 (aPTT) 延长,因子VIII (FVIII) 活性严重降低 (4%),FVIII 抑制剂定位呈阳性 (3.1 BU).
- 图像检查证实了肌内血瘤.
- 患者在治疗开始后24小时内经历了接近正常的aPTT和完全症状消失的快速临床改善,FVIII活动正常化,并在一个月的随访中没有复发.
结论:
- 这一案例强调了在没有典型危险因素或粘膜皮肤出血的情况下,在患有不明原因出血的患者中考虑获得的血友病A的重要性.
- 早期和向的免疫抑制疗法对于快速消除抑制剂和在获得的血友病A中取得有利结果至关重要.
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