丙戊酸诱发的罕见X因子缺乏症病例。
A rare case of factor X deficiency induced by valproic acid.
作者信息
Rigon Pierre-Antonin, Ernest Vincent
机构信息
Aix-Marseille Université, Assistance Publique Hôpitaux de Marseille, Hôpital Conception, Centre de Néphrologie et Transplantation Rénale, Marseille, France.
Laboratoire d'Hématologie, Hôpital de la Timone, Assistance Publique-Hôpitaux de Marseille, Marseille, France.
出版信息
Res Pract Thromb Haemost. 2025 Mar 3;9(2):102721. doi: 10.1016/j.rpth.2025.102721. eCollection 2025 Feb.
BACKGROUND
Factor X (FX) deficiency (FXD) significantly disrupts coagulation, potentially leading to severe bleeding. While inherited FXD is rare, with a prevalence of 1 in 500,000, acquired FXD is also uncommon and frequently linked to conditions such as light-chain amyloidosis. In rare cases, certain medications can cause FXD.
KEY CLINICAL QUESTION
Here, we present a rare case of acquired FXD induced by valproic acid (VPA). This deficiency is associated with the presence of anti-FX antibodies.
CLINICAL APPROACH
A 65-year-old man undergoing treatment for various conditions, including chronic kidney disease and type 2 diabetes, developed severe FXD (activity <2 U/L) following VPA administration for epilepsy. During FXD, the patient experienced significant bleeding episodes, necessitating FX replacement with prothrombin complex concentrate. Upon discontinuation of VPA, FX activity improved in 9 days, possibly suggesting a role of the drug in FXD. Interestingly, antibodies directed against FX have been identified.
CONCLUSION
This case emphasizes the necessity for clinicians to be vigilant of hemostasis disorders associated with VPA, even though such occurrences are rare.
背景
凝血因子X（FX）缺乏症（FXD）会严重破坏凝血功能，可能导致严重出血。虽然遗传性FXD很罕见，患病率为五十万分之一，但获得性FXD也不常见，且常与轻链淀粉样变等疾病相关。在罕见情况下，某些药物可导致FXD。
关键临床问题
在此，我们报告一例由丙戊酸（VPA）诱发的罕见获得性FXD病例。这种缺乏与抗FX抗体的存在有关。
临床处理方法
一名65岁男性正在接受包括慢性肾病和2型糖尿病在内的多种疾病治疗，在接受VPA治疗癫痫后出现严重FXD（活性<2 U/L）。在FXD期间，患者经历了严重的出血事件，需要用凝血酶原复合物浓缩物替代FX。停用VPA后，FX活性在9天内有所改善，这可能表明该药物在FXD中起作用。有趣的是，已发现针对FX的抗体。
结论
该病例强调，尽管此类情况罕见，但临床医生仍需警惕与VPA相关的止血障碍。
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