後産期出血の原因としての先天性第VII因子欠乏症：症例報告

Obstetric hemorrhage is one of the leading causes of maternal morbidity and mortality worldwide. However, coagulation disorders as an etiology are rare, with a prevalence of less than 1 %. This report concerns the case of a 24-year-old patient with no previous history of bleeding who presented with postpartum hemorrhage at 38.6 weeks of pregnancy after a normal vaginal delivery. The hemorrhage was refractory to the use of uterotonics and compression maneuvers. In the absence of uterine atony, retained products of conception, or obvious trauma, the possibility of an underlying coagulopathy was considered. Initial studies showed an isolated prolongation of prothrombin time, with activated partial thromboplastin time within normal parameters. A correction test with a normal plasma pool was performed, which showed complete correction of prothrombin time. Specific factor quantification confirmed a severe and isolated factor VII deficiency.