Coagulation Conundrum : A Case of an Elevated INR Secondary to Combined Factor V and VII Deficiency

Congenital factor V (FV) and factor VII (FVII) deficiency is a rare coagulopathy, caused by two distinct independently segregating genetic defects. A 54-year-old female presented to hospital with non-ST elevation myocardial infarction. Workup revealed an elevated international normalized ratio (INR) of 2.0 (normal range 0.8–1.2). Thrombin time (TT), partial thromboplastin time (PTT), and Clauss fibrinogen were normal. Her only prior bleeding event was a lower gastrointestinal (GI) bleed occurring during antiplatelet therapy. She was not on chronic anticoagulant therapy. Her INR did not correct to normal despite 30 mg of oral/intravenous vitamin K. Hepatic synthetic function was normal arguing against an acquired coagulopathy of liver disease. Coagulation factor levels revealed reduced levels of FV and FVII. A combined congenital FV and FVII deficiency was felt to be the most likely diagnosis. During her hospital stay, she underwent coronary artery bypass grafting with perioperative recombinant FVIIa and is expected to have confirmatory genetic testing. Our case demonstrates the value of a thorough bleeding history and the importance of following up patients with such a history.