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一个非中和性获得因子V抑制剂模仿缺陷的案例报告:诊断挑战和治疗影响

Shreyas Kalantri¹, Pranali Pachika¹, Shiva Balasubramanian²

¹Division of Hematology and Oncology, Brown Cancer Center, University of Louisville, Louisville, Kentucky, USA, louisville.edu.

Case reports in hematology

|January 12, 2026

概括

由非中和抑制剂引起的罕见获得的V因子缺乏症提出了诊断挑战. 用Rituximab和IVIG治疗使V因子水平和凝血时间正常化,证明了有效的管理.

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:

获得的V因子 (FV) 缺乏与抑制剂是一种罕见的凝血病.
它带来了重大的诊断和治疗挑战.

研究的目的:

报告由于非中和抑制剂而导致的获得的FV缺陷病例.
为了说明诊断的复杂性和成功的治疗策略.

主要方法:

一个81岁的男性患有严重的凝血病和低FV活动的病例报告.
诊断工作包括混合研究和标准疗法的评估 (FFP,rFVIIa).
治疗包括血小板输血,利图西马布和静脉注射免疫球蛋白 (IVIG).

主要成果:

最初的测试表明缺陷,但标准疗法失败了.
怀疑是一种非中和抑制剂,增加了FV清除.
治疗Rituximab和IVIG使FV水平正常化,并纠正PT/aPTT.
患者实现了临床改善,并维持了正常凝血与维护免疫抑制.

结论:

非中和的FV抑制剂通过加速FV清除,造成诊断困难.
整合临床和实验室发现对于量身定制的治疗至关重要.
利图西马布和IVIG可以有效地治疗这种罕见的凝血病.

关键词:

这是一种V因子抑制剂.获得的V因子缺乏症.凝血病是一种凝血病.流血症流血症流血症流血症免疫抑制疗法是一种免疫抑制疗法.静脉注射免疫球蛋白 (IVIG) 是一种没有中和抗体的抗体.血小板输血输血血小板输血这是一种罕见的出血障碍.这就是Rituximab.

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