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HEMOPHILIA.ORG > BLEEDING DISORDERS INFO CENTER > TYPES >
FACTOR VII DEFICIENCY


Factor VII Deficiency
(Proconvertin or Stable Factor)

What Is It?
This extremely rare disorder can be inherited or acquired by people who do not have hemophilia who take Coumadin, a drug used to inhibit blood clotting.

With this disorder, bleeding can vary from mild to severe within the same person over time. Bleeding doesn't always correspond with the severity of the deficiency shown in blood tests. A history of bleeding may occur in infancy or childhood. Gastrointestinal and central nervous system bleeding can also occur.

This disorder occurs in one in 500,000 males and females. Congenital factor VII deficiency should be distinguished from acquired factor VII deficiency, which may result from liver disease, vitamin K deficiency or other malabsorption conditions.

When levels of the factor are less than 1% of normal, bleeding can be severe. The trauma of birth may cause bleeding in the head of a newborn. Circumcision may cause prolonged bleeding. Children and adults may suffer bleeding from nose, gums or gastrointestinal tract, and women may suffer excessive menstrual bleeding.

The probable outcome is good with proper treatment.

Inheritance Pattern
The disorder is not sex-linked as is hemophilia. It affects both males and females with equal frequency. It is also autosomal recessive, which means that if the clotting defect is inherited from a parent, the child will be a genetic carrier of the condition, but may or may not have symptoms. Those who have inherited a defective factor VII gene from only one parent will usually have only moderate levels of the factor but no symptoms.

Symptoms and Diagnosis
Severity of bleeding episodes depends on the degree of the severity of the defect. Symptoms include:
• bleeding of mucous membranes
• spontaneous nosebleeds
• excessive bruising
• prolonged menstrual bleeding
• bleeding into muscles
• bleeding into joints

Diagnosis is made by testing for factor VII in the blood. Signs and tests include:
• prolonged prothrombin time
• normal partial thromboplastin time
• decreased factor VII assay

Treatments
Bleeding episodes can be controlled with normal plasma or concentrates containing factor VII. Severe bleeding may be treated with fresh frozen plasma or Prothrombin complex concentrates (PCCs). Because the life span of infused factor VII is very short (two to four hours), patients may require treatment every two to six hours for severe bleeding or surgery. Menstrual bleeding can be controlled by the use of oral contraceptives.

Note that factor VII concentrate is sold only in Europe, but is available in the US on a compassionate basis from the manufacturer (Immuno Ag).

The National Hemophilia Foundation's Medical and Scientific Advisory Council (MASAC) made recommendations for treatment of Factor VII deficiency in November of 1999. They include:
• Recombinant factor VIIa (NovoSeven), which was recently licensed in the US and is produced by baby hamster kidney cells. No human albumin is used.
• Prothrombin complex concentrates (PCCs) can be used, but these products vary considerably in the amount of factors they contain.
• Fresh frozen plasma can be used as along as it is processed to reduce the risk of viral infection.

Complications
These can include hemorrhages, strokes or other neurological problems related to central nervous system bleeding. Fatal intracranial bleeding caused by birth trauma has occurred. Menstrual bleeding may also be severe.

Disclaimer
The information contained on the NHF web site is provided for your general information only. NHF does not give medical advice or engage in the practice of medicine. NHF under no circumstances recommends particular treatment for specific individuals and in all cases recommends that you consult your physician or local treatment center before pursuing any course of treatment.
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