Factor VII deficiency is an inherited disorder in which a lack (deficiency) of plasma protein factor VII leads to abnormal bleeding.
Extrinsic factor deficiency
When you bleed, the body launches a series of reactions that help the blood clot. This is called the coagulation cascade. The process involves special proteins called coagulation or clotting factors. When one or more of these clotting factors are missing, there is usually a higher chance of bleeding.
This disorder occurs when the body does not have enough factor VII, an important blood clotting protein. Most often a deficiency of factor VII is caused by:
It is very rare to be born with factor VII deficiency that is due to the body's inability to make working factor VII.
Diagnosing a bleeding disorder is important so that the doctor can take extra care if you need surgery, and can test or warn other family members who might be affected.
Patients can control bleeding episodes by receiving normal plasma, concentrates of factor VII, or genetically produced (recombinant) factor VII through a vein (intravenous). People need frequent treatment during bleeding episodes because factor VII does not last for long inside the body. A form of factor VII called NovoSeven can also be used.
If a lack of vitamin K is causing the disorder, you can take vitamin K by mouth, through injections under the skin, or through a vein (intravenously).
You can often help the stress of illness by joining a support group where members share common experiences and problems. See hemophilia - resources.
The outcome can be good with proper treatment.
This is a life-long disorder if you get it from your parents. If it is caused by liver disease, the outcome depends on how well your liver problem can be treated.
Go to the emergency room or call the local emergency number (such as 911) if you have severe, unexplained bleeding.
Genetic counseling may be helpful for disorders that start at birth (congenital). Taking vitamin K can help if you have a vitamin K deficiency.
Gailani d, Neff AT. Rare coagulation factor deficiencies. In: Hoffman R, Benz EJ Jr, Shattil SJ, et al, eds. Hoffman Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Churchill Livingstone Elsevier;2008:chap 127.
Kessler C. Hemorrhagic disorders: coagulation factor deficiencies. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa:Saunders Elsevier;2007:chap 180.