Factor VII Deficiency

An estimated 1 in 500,000 people have factor VII deficiency, though some experts believe it might be more common than that. It is inherited in an autosomal recessive fashion, which means it affects men and women equally.

The factor VII protein is part of the cascade of clotting factors that form the chain leading to a protective blood clot. Factor VII deficiency is usually severe. In fact patients with less than 1% factor VII activity experience similar symptoms to hemophilia. People with severe factor VII are prone to joint bleeds. In addition to spontaneous nosebleeds, people can experience bleeds in the stomach, intestines and urinary tract. Head bleeds and muscle bleeds have also been reported. Women can have severe menorrhagia.

Diagnosis is made through activated partial thromboplastin time (aPTT) test, prothrombin time (PT) test and thrombin time (TT) test. Diagnosis can be confirmed with a factor VII assay. There have been instances of factor VII deficiencies in combination with deficiencies of factors II, IX and X.

Treatment

Prothrombin complex concentrates (PCCs) can be used to treat factor VII deficiency. However, the amount of factor VII contained in PCCs varies considerably among these products. Not only is there a marked difference in factor content between the different commercial preparations, but factor content can also vary between product lots produced by the same manufacturer. These products are not available in all countries but may be available in yours.

Patients with factor VII deficiency can also be treated with fresh frozen plasma (FFP). However, volume constraints may limit the amount of FFP that can be used. In addition, there have been cases of thrombosis reported in people with factor VII deficiency.

Novo Nordisk^® received FDA approval for a new usage indication of a recombinant factor VIIa product to treat bleeding episodes in patients with factor VII deficiency.

Novo Nordisk^® is a registered trademark of Novo Nordisk Pharmaceuticals Industries Inc.