Factor XII (Hageman factor) deficiency


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Definition

Factor XII deficiency is an inherited disorder that affects a protein (factor XII) involved in blood clotting.


Causes, incidence, and risk factors

When you bleed, the body launches a series of reactions that help the blood clot. This is called the coagulation cascade. The process involves special proteins called coagulation factors. (Factor XII is a coagulation factor in this series of reactions.)

Each factor has a reaction that triggers the next reaction. The final product of the coagulation cascade is the blood clot. When one or more of these clotting factors are missing, there is usually a higher chance of bleeding.

A lack of factor XII does not cause the affected person to bleed abnormally, but the blood takes longer than normal to clot in a test tube.

Factor XII deficiency is a rare inherited disorder.


References

Gailani D, Neff AT. Rare coagulation factor deficiencies. In: Hoffman R, Benz EJ Jr, Shattil SJ, et al, eds. Hoffman Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Churchill Livingstone Elsevier;2008:chap 127.

Kessler C. Hemorrhagic disorders: Coagulation factor deficiencies. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 180.


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Review Date: 2/28/2011
Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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Last Updated 5/17/2011
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