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Factor X deficiency

Stuart-Prower deficiency

 

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

Causes

 

When you bleed, a series of reactions take place in the body that helps blood clots form. This process is called the coagulation cascade. It involves special proteins called coagulation, or clotting, factors. You may have a higher chance of excess bleeding if one or more of these factors are missing or are not functioning like they should.

Factor X is one such coagulation factor. Factor X deficiency is often caused by an inherited defect in the factor X gene. This is called inherited factor X deficiency. Bleeding ranges from mild to severe depending on how severe the deficiency is.

Factor X deficiency can also be due to another condition or use of certain medicines. This is called acquired factor X deficiency. Acquired factor X deficiency is common. It can be caused by:

  • Lack of vitamin K (some newborns are born with vitamin K deficiency)
  • Buildup of abnormal proteins in the tissues and organs ( amyloidosis )
  • Severe liver disease
  • Use of medicines that prevent clotting (anticoagulants such as warfarin)

Women with factor X deficiency may first be diagnosed when they have very heavy menstrual bleeding and bleeding after childbirth. The condition may be first noticed in newborn boys if they have bleeding that lasts longer than normal after circumcision.

 

Symptoms

 

Symptoms may include any of the following:

  • Bleeding into the joints
  • Bleeding into the muscles
  • Bruising easily
  • Heavy menstrual bleeding
  • Mucus membrane bleeding
  • Nosebleeds that do not stop easily
  • Umbilical cord bleeding after birth

 

Exams and Tests

 

Tests that may be done include:

  • Low factor X activity
  • Normal thrombin time
  • Prolonged partial thromboplastin time (PTT)
  • Prolonged prothrombin time (PT)

 

Treatment

 

Bleeding can be controlled by getting intravenous (IV) infusions of plasma or concentrates of clotting factors. If you lack vitamin K, you can take vitamin K by mouth, through injections under the skin, or through a vein (intravenously).

If you have this bleeding disorder, be sure to:

  • Tell your health care providers before you have any kind of procedure, including surgery and dental work.
  • Tell your family members because they may have the same disorder but do not know it yet.

 

Support Groups

 

Find out more about bleeding disorders through these groups:

American Society of Hematology -- www.hematology.org/Patients/Blood-Disorders.aspx

National Hemophilia Foundation: Other Factor Deficiencies -- www.hemophilia.org/Bleeding-Disorders/Types-of-Bleeding-Disorders/Other-Factor-Deficiencies

Victory for Women with Blood Disorders -- www.hemophilia.org/Community-Resources/Women-with-Bleeding-Disorders/Victory-for-Women-with-Blood-Disorders

 

Outlook (Prognosis)

 

The outcome is good if the condition is mild or you get treatment.

Inherited factor X deficiency is a lifelong condition.

The outlook for acquired factor X deficiency depends on the cause. If it is caused by liver disease, the outcome depends on how well your liver disease can be treated. Taking vitamin K supplements will treat vitamin K deficiency. If the disorder is caused by amyloidosis, there are several treatment options. Your doctor can tell you more.

 

Possible Complications

 

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

 

When to Contact a Medical Professional

 

Get emergency medical help if you have an unexplained or severe loss of blood.

 

Prevention

 

There is no known prevention for inherited factor X deficiency. When a lack of vitamin K is the cause, using vitamin K can help.

 

 

References

Gailani D, Neff AT. Rare coagulation factor deficiencies In: Hoffman R, Benz EJ Jr, Silberstein LE, Heslop HE, Weitz JI, Anastasi JI, eds. Hematology: Basic Principles and Practice . 6th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 139.

Ragni MV. Hemorrhagic disorders: coagulation factor deficiencies. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine . 24th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 177.

 
  • Blood clot formation - illustration

    Blood clotting normally occurs when there is damage to a blood vessel. Platelets immediately begin to adhere to the cut edges of the vessel and release chemicals to attract even more platelets. A platelet plug is formed, and the external bleeding stops. Next, small molecules, called clotting factors, cause strands of blood-borne materials, called fibrin, to stick together and seal the inside of the wound. Eventually, the cut blood vessel heals and the blood clot dissolves after a few days.

    Blood clot formation

    illustration

  • Blood clots - illustration

    Blood clots (fibrin clots) are the clumps that result when blood coagulates.

    Blood clots

    illustration

    • Blood clot formation - illustration

      Blood clotting normally occurs when there is damage to a blood vessel. Platelets immediately begin to adhere to the cut edges of the vessel and release chemicals to attract even more platelets. A platelet plug is formed, and the external bleeding stops. Next, small molecules, called clotting factors, cause strands of blood-borne materials, called fibrin, to stick together and seal the inside of the wound. Eventually, the cut blood vessel heals and the blood clot dissolves after a few days.

      Blood clot formation

      illustration

    • Blood clots - illustration

      Blood clots (fibrin clots) are the clumps that result when blood coagulates.

      Blood clots

      illustration

    A Closer Look

     

      Self Care

       

        Tests for Factor X deficiency

         

           

          Review Date: 1/22/2015

          Reviewed By: Rita Nanda, MD, Assistant Professor of Medicine, Section of Hematology/Oncology, University of Chicago Medicine, Chicago, IL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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