Health Encyclopedia

Factor X deficiency

Definition

Factor X deficiency is a disorder caused by too little of a protein called factor X in the blood. It leads to problems with blood clotting (coagulation).

Alternative Names

Stuart-Prower deficiency

Causes, incidence, and risk factors

When you bleed, the body launches a series of activities that help the blood clot. This is called the coagulation cascade. The process involves special proteins called coagulation factors. (Factor X is a coagulation factor.) Each factor's reaction triggers the next reaction. The final product of the coagulation cascade is the blood clot.

When certain coagulation factors are missing, the chain reaction does not take place normally. Factor X deficiency is often caused by a defect of the factor X gene that is passed through families (inherited). Bleeding ranges from mild to severe. Another cause of factor X deficiency is amyloidosis (a disorder in which protein fibers that can't dissolve deposit in tissues and organs and affect their function).

Women with this condition may have very heavy menstrual bleeding and bleeding after delivery. Newborn boys with the condition may have longer-than-normal bleeding after circumcision.

Factor X deficiency affects 1 out of every 500,000 people.

Symptoms

Nose bleeds
The loss of blood into joints
Muscle bleeding
Mucous membrane bleeding

Signs and tests

Treatment

Treatment for the bleeding disorder is to get fresh frozen plasma or factor X concentrates into the blood during sudden (acute) episodes or before surgery. If the disorder is caused by amyloidosis, surgery is done to remove the spleen.

Support Groups

You can ease the stress of illness by joining a support group where members share common experiences and problems. See hemophilia - resources.

Expectations (prognosis)

The outcome is usually good with mild disease or treatment.

Complications

Severe bleeding or sudden loss of blood (hemorrhage) can occur. The joints may get deformed in severe disease from many bleeds.

Calling your health care provider

Call your health care provider, go to the emergency room, or call the local emergency number (such as 911) if you have an unexplained or severe loss of blood.

Prevention

This is a rare disorder that runs in families. There is no known way to prevent it.

References

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

Sucker C, Hetzel GR, Grabensee B, Stockschlaeder M, Scharf RE. Amyloidosis and Bleeding: Pathophysiology, Diagnosis, and Therapy. Am J Kidney Dis. June 2006;47:947-955.

Takabe K, Holman PR, Herbst KD, Glass CA, Bouvet M. Successful perioperative management of factor X deficiency associated with primary amyloidosis. J Gastrointest Surg. March 2004;8:358-362.

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

ADAM Quality A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is the first of its kind, requiring compliance with 53 standards of quality and accountability, verified by independent audit. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial process. A.D.A.M. is also a founding member of Hi-Ethics (www.hiethics.com) and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The Agency for Health Care Administration (Agency) and this website do not claim the information on, or referred to by, this site is error free. This site may include links to websites of other government agencies or private groups. Our Agency and this website do not control such sites and are not responsible for their content. Reference to or links to any other group, product, service, or information does not mean our Agency or this website approves of that group, product, service, or information.

Additionally, while health information provided through this website may be a valuable resource for the public, it is not designed to offer medical advice. Talk with your doctor about medical care questions you may have.