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Congenital afibrinogenemia

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Definition:

Congenital afibrinogenemia is a rare, inherited blood disorder in which the blood does not clot normally. It occurs when you are missing or have a problem with a protein called fibrinogen, which is needed for the blood to clot.

Causes, incidence, and risk factors:

This rare disease is caused by an abnormal gene that must be passed down from both parents. There may be either a lack of fibrinogen or a defect in the functioning of available fibrinogen. This condition can occur in both sexes. The main risk factor is a family history of bleeding disorders.

References:

Hoffman R, Benz E, Shattil S, Furie B, Cohen H. Hematology: Basic Principles and Practice . 4th ed. Philadelphia, Pa: Churchill Livingstone, 2004.

Asselta R, Duga S, Tenchini ML. The molecular basis of quantitative fibrinogen disorders. J Thromb Haemost . October 2006;4:2115-2129.

• Review Date: 3/13/2007
• Reviewed By: Mark Levin, M.D., Hematologist and Oncologist, Newark, NJ. Review provided by VeriMed Healthcare Network.

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