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Inherited factor VII deficiency is an autosomal recessive coagulation disorder with broad range of bleeding manifestations. The association between bleeding and absolute factor VII level is poor. Usually, the bleeding is associated with FVII levels of less than 1% of the normal value. Factor VII deficiency is associated with prolongation of prothrombin time only with normal activated partial thromboplastin time. Approximately 66 pregnant women have been reported with factor VII deficiency so far in English literature. We hereby, report 2 cases along with the review of literature of Factor VII deficiency during pregnancy. Our patients were diagnosed to have factor VII deficiency after deranged coagulogram with factor VII level of < 1% and 17.1% respectively, however could be managed by fresh frozen plasma only in first case and fresh frozen plasma & factor VII concentrate in second case successfully. Coagulogram is a simple, easily available, affordable and lifesaving investigation to detect this deficiency in pregnancy.
Inherited factor VII deficiency is an autosomal recessive coagulation disorder with broad range of bleeding manifestations. The association between bleeding and absolute factor VII level is poor. Usually, the bleeding is associated with FVII levels of less than 1% of the normal value. Factor VII deficiency is associated with prolongation of prothrombin time only with normal activated partial thromboplastin time. Approximately 66 pregnant women have been reported with factor VII deficiency so far in English literature. We hereby, report 2 cases along with the review of literature of Factor VII deficiency during pregnancy. Our patients were diagnosed to have factor VII deficiency after deranged coagulogram with factor VII level of < 1% and 17.1% respectively, however could be managed by fresh frozen plasma only in first case and fresh frozen plasma & factor VII concentrate in second case successfully. Coagulogram is a simple, easily available, affordable and lifesaving investigation to detect this deficiency in pregnancy.
This paper aims to study antepartum and postpartum bleeding manifestations of patients with factor VII (FVII) deficiency, their management, and feto-maternal outcomes, to establish danger signs and management protocols. We describe a case series of nine pregnancies in four patients with FVII deficiency diagnosed at a tertiary care referral center in India between 2012 and 2023. Out of nine pregnancies, six had cesarean deliveries, two had vaginal deliveries, and one had dilatation and curettage for unwanted pregnancies. One out of nine pregnancies (11.11%) with an unknown FVII deficiency had antepartum hemorrhage (abruption) necessitating multiple transfusions, ICU stay, and neonatal loss. Three patients with no prior history of obstetric hemorrhage were diagnosed with severe deficiencies and received prophylactic recombinant FVII preoperatively, averting the potential loss of lives. In patients with no history of bleeding, no hemorrhage was reported with or without prophylaxis while 33.33% of hemorrhage was reported in patients with a history of bleeding. Factors like the history of bleeding, FVII levels, mode of delivery, and other risk factors for hemorrhage should all be considered to predict the risk of bleeding in delivery. Cesarean is a surgical procedure, and prophylactic use of recombinant FVII concentrate (rFVIIa) should be considered.
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