Factor X deficiencyDefinition: 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 reactions 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. This is called inherited factor X deficiency. Bleeding ranges from mild to severe.
Factor X deficiency may also develop due to another condition or use of medications. This is called aquired factor X deficiency. Acquired factor X deficiency is common. It may be caused by a lack of vitamin K, amyloidosis , severe liver disease, and use of drugs that prevent clotting (anticoagulants such as warfarin or coumadin). Some newborns are born with vitamin K deficiency. See: Vitamin k deficiency bleeding in newborns
Women with factor X deficiency may have very heavy menstrual bleeding and bleeding after delivery. Newborn boys with the condition may have longer-than-normal bleeding after circumcision.
Inherited factor X deficiency affects 1 out of every 500,000 people.
Treatment: Treatment for the bleeding disorder involves infusions of fresh frozen plasma or factor X concentrates into the blood during sudden (acute ) episodes or before surgery.
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.
Inherited factor X deficiency is a life-long condition.
The outlook for acquired factor X deficiency depends on the underlying cause. If it is caused by liver disease, the outcome depends on how well your liver disease is controlled. Taking vitamin K supplements will treat vitamin K deficiency. If the disorder is caused by amyloidosis, surgery is done to remove the spleen.
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: Inherited factor X deficiency is rare. There is no known prevention.
References: Kessler C. Hemorrhagic disorders: Coagulation factor deficiencies. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 180.
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.
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