ABSTRACT
Factor VII deficiency is a rare hereditary coagulation disorder with an incidence estimated at 1 in 500,000 individuals. In this report, we describe the 13th case in pregnancy. The diagnosis of severe factor VII deficiency (factor VII level <5%) was established at 10 weeks' gestation after initial laboratory testing showed a markedly prolonged prothrombin time and a normal activated partial thromboplastin time. There was a history of two preterm deliveries, but there was no evidence of previous bleeding manifestations. Antenatal progress of the index pregnancy was unremarkable. Prophylactic treatment with fresh frozen plasma was started at the onset of labor and the patient had a vaginal delivery of a live girl at 36 weeks' gestation. There was no post-partum hemorrhage and mother and newborn were discharged in good condition. The patient's postpartum level of factor VII remained undetectable. Two aspects are outlined: the absence of any significant increase in factor VII clotting activity during this pregnancy and the need to give replacement therapy at labor in patients with severe factor VII deficiency to decrease the risk of postpartum hemorrhage.
Keywords
Factor VII deficiency - inherited coagulation disorders - postpartum hemorrhage - pregnancy
