Factor VII deficiency

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Blood clot formation

Factor VII deficiency

Definition:

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:

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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 reactionsinvolving these coagulation factors takes place very rapidly, forming 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.