关键词: 因子Ⅴ, 缺乏症, 妊娠, 产后出血, 病例报告

Abstract:

Coagulation factor Ⅴ(FⅤ) deficiency is a rare genetic disorder that is primarily characterized by decreased FⅤ activity(FⅤC) and extensive clinical bleeding symptoms. Pregnancy combined with FⅤ deficiency is even more rare, and the risk of poor prognosis is higher. In this article, we report a case of severe homozygote hereditary FⅤ deficiency and postpartum hemorrhage occurring simultaneously. The patient was treated with ongoing high-dose blood product transfusions after being admitted to the hospital with a clear diagnosis of FⅤ deficiency, but the FⅤC did not improve adequately. Later, a live infant was delivered by cesarean section for acute fetal distress, bilateral uterine artery embolization was done for combined postpartum hemorrhage, and postoperative review revealed that the FⅤC had not returned to the normal level, and the patient was discharged with stable condition after symptomatic treatment. We now go over the medical history, the clinical course of this case, and the pertinent literature, with the aim of increasing clinicians′ understanding of this disease and accumulate more clinical experience.

Key words: Factor Ⅴ, Deficiency diseases, Pregnancy, Postpartum hemorrhage, Case reports