What Is Factor VII Deficiency? 

Factor VII (proconvertin) deficiency is an inherited bleeding disorder caused by a defect or lack of a protein in the blood (factor VII) that helps blood clot. The disorder is rare, affecting one in 500,000 people. It is passed from parent to child and is an autosomal recessive disorder, meaning that both parents must carry the defective gene in order to pass it on to their child and that it affects both males and females.  The gene is found on chromosome 13.

Factor VII deficiency may also be acquired through liver disease, low levels of vitamin K, or from other problems with absorption. The acquired form of the disorder can also develop in people who take coumadin, a medication that prevents blood clotting.

Symptoms

People with factor VII deficiency may experience:

• bleeding in the head (in newborns)
• heavy bleeding at circumcision
• nose bleeds
• bleeding from the gums
• heavy or prolonged menstrual bleeding (menorrhagia)
• bleeding from the gut (gastrointestinal bleeding)

Diagnosis

Factor VII deficiency is diagnosed by a variety of blood tests to see if there is a bleeding disorder and, if so, to determine which one it is.

Treatment

Factor VII deficiency is treated with:

• recombinant VIIa concentrates
• factor VII concentrates
• prothrombin complex concentrates containing factor VII
• fresh frozen plasma (FFP)

Content reviewed by Dr. Paula Bolton-Maggs
Consultant Haemotologist
Manchester Haemophilia Comprehensive Care
Manchester Royal Infirmary
Manchester, UK.

Last updated December 2007