Balluet Rémi, Bourguignon Alexane, Geay-Baillat Marie-Odile, Le Quellec Sandra
Service d'hématologie biologique, Centre de biologie et de pathologie Sud, Hospices civils de Lyon, France.
Service d'hématologie biologique, Centre de biologie et de pathologie Est, Hospices civils de Lyon, France, Unité d'hémostase clinique, Hôpital cardiologique Louis Pradel, Hospices civils de Lyon, France.
Ann Biol Clin (Paris). 2020 Apr 1;78(2):198-200. doi: 10.1684/abc.2020.1545.
Factor VII deficiency is the most common of the rare coagulation deficiencies. A hemorrhagic syndrome may occur in patients with FVII deficiency below 20%, although no correlation exist between the plasma FVII activity level (FVII:C) and the bleeding risk. Therefore, the management of surgery in patients with FVII deficiency remains challenging. Laboratory monitoring of FVII:C level may be helpful but should be interpreted with caution, because the dosage of FVII:C level may vary depending on the origin of the thromboplastin used. Herein, we report the case of the management of a woman who had been fortuitously diagnosed during pregnancy with FVII deficiency due to FVII variant Padua, which have induced discrepant results between two different laboratories.
因子 VII 缺乏症是最常见的罕见凝血因子缺乏症。血浆 FVII 活性水平低于 20% 的 FVII 缺乏症患者可能会出现出血综合征，尽管血浆 FVII 活性水平（FVII:C）与出血风险之间不存在相关性。因此，FVII 缺乏症患者的手术管理仍然具有挑战性。FVII:C 水平的实验室监测可能会有所帮助，但应谨慎解读，因为 FVII:C 水平的测定值可能因所用凝血活酶的来源而异。在此，我们报告了一名因 FVII 帕多瓦变异体在孕期偶然诊断为 FVII 缺乏症的女性的管理病例，该变异体导致了两个不同实验室之间的结果差异。
