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Factor XII (Hageman factor) deficiency
Definition
Factor XII deficiency is an inherited disorder that affects a protein (factor XII) involved in blood clotting.
Causes
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.
Symptoms
There are usually no symptoms.
Exams and Tests
Factor XII deficiency is usually found when clotting tests are done for routine screening.
Tests may include:
- Factor XII assay
- Partial thromboplastin time (PTT)
Treatment
Treatment is usually not needed.
Outlook (Prognosis)
The outcome is expected to be good without treatment.
Possible Complications
There are usually no complications.
When to Contact a Medical Professional
The health care provider usually discovers this condition after noticing prolonged clotting in the process of running other laboratory tests.
Prevention
This is an inherited disorder. There is no known way to prevent it.
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.
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.
