Factor VII deficiency

Factor VII deficiency is an inherited disorder that causes abnormal blood clotting (coagulation), resulting from a deficiency of the plasma protein Factor VII.

Alternative Names

Extrinsic factor deficiency

Causes, incidence, and risk factors

This disorder is caused by an inherited deficiency of Factor VII, an important clotting protein. Normal blood coagulation is a complex process involving as many as 20 different plasma proteins , which are known as blood coagulation factors. A series of complex chemical reactions using these factors takes place very rapidly to form an insoluble protein called fibrin that stops bleeding . When certain coagulation factors are deficient or missing, the chain reaction does not take place normally. In this disorder, bleeding can vary from mild to severe within the same person over time. A history of bleeding may occur in infancy or childhood. Gastrointestinal and central nervous system bleeding can occur. The risk factor is a family history of bleeding. The incidence is 1 in 500,000.

Signs and tests

  • Prolonged prothrombin time
  • Normal
  • partial thromboplastin time
  • Decreased plasma
  • Factor VII activity

    Treatment

    Bleeding episodes can be controlled with normal plasma, concentrates containing Factor VII, or recombinant Factor VII. During bleeding episodes, frequent treatment is needed because the life span of Factor VII is short. Menstrual bleeding can be controlled by the use of oral contraceptives.

    Support groups

    The stress of illness can often be helped by joining a support group where members share common experiences and problems. See hemophilia - support group .

    Expectations (prognosis)

    The probable outcome is good with proper treatment.

    Complications

  • Hemorrhage
  • Stroke
  • or other neurological problems related to
  • central nervous system bleeding
  • Joint deformities may develop in severe cases due to recurrent bleeds
  • Calling your health care provider

    Go to the emergency room or call the local emergency number (such as 911) if you have severe, unexplained bleeding .

    Prevention

    This is an inherited disorder. There is no known prevention.

    Treatment Options – Sorted by Soonest Available

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