Factor X deficiency
Key points
- Factor X deficiency is a disorder that causes abnormal blood clotting (coagulation), resulting from a shortage of a plasma protein called factor X.
- Normal blood coagulation is a complex process involving as many as 20 different plasma proteins, which are known as blood coagulation factors.
- Treatment The bleeding disorder is corrected by infusions of fresh frozen plasma or factor X concentrates during acute episodes or in preparation for surgery.
- The stress of illness can often be helped by joining a support group where members share common experiences and problems.
Alternative names
Stuart-Prower deficiency
Definition
Factor X deficiency is a disorder that causes abnormal blood clotting (coagulation), resulting from a shortage of a plasma protein called factor X.
Causes, incidence, and risk factors
Normal blood coagulation is a complex process involving as many as 20 different plasma proteins, which are known as blood coagulation factors. A series of complex chemical reactions using these factors takes place very rapidly to form an insoluble protein called fibrin that stops bleeding.
When certain coagulation factors are deficient or missing, the chain reaction does not take place normally. Factor X deficiency is often caused by an inherited defect of the factor X gene, and bleeding ranges from mild to severe. Another cause of factor X deficiency is amyloidosis (a disorder in which insoluble protein fibers deposit in tissues and organs, impairing their function).
Women with this condition may have severe menstrual bleeding and bleeding after delivery. Newborn boys with the condition may have prolonged bleeding after circumcision.
The incidence is 1 out of 500,000.
Symptoms
- Nose bleeds
- The loss of blood into joints
- Muscle bleeding
- Mucous membrane bleeding
Signs and tests
- Prolonged prothrombin time
- Prolonged partial thromboplastin time
- Low factor X activity
- Normal thrombin time
Treatment
The bleeding disorder is corrected by infusions of fresh frozen plasma or factor X concentrates during acute episodes or in preparation for surgery. Treatment for factor X deficiency related to amyloidosis involves surgical removal of the spleen.
Support Groups
The stress of illness can often be helped 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 with adequate therapy.
Complications
Severe bleeding or hemorrhage can occur. Joint deformities may result from repeated bleeds in severe disease.
Frequently asked questions
What is factor x deficiency?
Factor X deficiency is a disorder that causes abnormal blood clotting (coagulation), resulting from a shortage of a plasma protein called factor X.
What are the risk factors for factor x deficiency?
Normal blood coagulation is a complex process involving as many as 20 different plasma proteins, which are known as blood coagulation factors. A series of complex chemical reactions using these factors takes place very rapidly to form an insoluble protein called fibrin that stops bleeding. When certain coagulation factors are deficient or missing, the chain reaction does not take place normally. Factor X deficiency is often caused by an inherited defect of the factor X gene, and bleeding ranges from mild to severe. Another cause of factor X deficiency is amyloidosis (a disorder in which insoluble protein fibers deposit in tissues and organs, impairing their function).
What are the symptoms of factor x deficiency?
- Nose bleeds
- The loss of blood into joints
- Muscle bleeding
- Mucous membrane bleeding
What is the outlook for factor x deficiency?
The outcome is usually good with mild disease or with adequate therapy.
Written and revised by
David Cohen, M.D.
Our doctors write and review articles for accuracy and keep them up to date.
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