Factor VII Deficiency

Factor VII deficiency is an inherited bleeding disorder that is caused by a problem with factor VII. Because the body produces less factor VII than it should, or because the factor VII is not working properly, the clotting reaction is blocked prematurely and the blood clot does not form.

Factor VII deficiency is an autosomal recessive disorder, which means that both parents must carry the defective gene in order to pass it on to their child. It also means that the disorder affects both males and females. Factor VII deficiency is very rare, but like all autosomal recessive disorders, it is found more frequently in areas of the world where marriage between close relatives is common.

Factor VII deficiency may be inherited with other factor deficiencies. It can also be acquired later in life as a result of liver disease, vitamin K deficiency, or certain medications such as the blood-thinning drug Coumadin®.

Symptoms

The symptoms of factor VII deficiency are different for everyone. As a general rule, the less factor VII a person has in his/her blood, the more frequent and/or severe the symptoms. People with very low levels of factor VII can have very serious symptoms.

Common symptoms

  • Nosebleeds (epistaxis)
  • Easy bruising
  • Heavy or prolonged menstrual bleeding (menorrhagia)
  • Bleeding in the mouth, particularly after dental surgery or tooth extraction
  • Bleeding in the head (newborns)
  • Heavy bleeding at circumcision

 

Other reported symptoms

  • Bleeding in the gut (gastrointestinal bleeding)
  • Bleeding into joints (hemarthrosis)
  • Muscle bleeds
  • Bleeding in the central nervous system (the brain and spinal cord)
  • Abnormal bleeding during or after injury, surgery, or childbirth

 

Rare symptoms

  • Blood in the urine (haematuria)
  • Bleeding from the umbilical cord stump at birth

 

Treatment

There are several treatments available for factor VII deficiency.

  • Recombinant factor VIIa concentrates (rFVIIa)
  • Factor VII concentrate
  • Prothrombin Complex Concentrate (PCC) containing factor VII
  • Fresh Frozen Plasma (FFP)

 

Excessive menstrual bleeding in women with factor VII deficiency may be controlled with hormonal contraceptives (birth control pills) or antifibrinolytic drugs.

Diagnosis

Factor VII deficiency is diagnosed by a variety of blood tests that should be performed by a specialist at your Comprehensive Care Centre.

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