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Factor VII deficiency

Extrinsic factor deficiency; Serum prothrombin conversion accelerator deficiency

 

Factor VII (seven) deficiency is a disorder caused by a lack of a protein called factor VII 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 VII is one such coagulation factor. Factor VII deficiency runs in families (inherited) and is very rare. Both parents must have the gene to pass the disorder on to their children. A family history of a bleeding disorder can be a risk factor.

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

  • Low vitamin K (some babies are born with vitamin K deficiency)
  • Severe liver disease
  • Use of medicines that prevent clotting (anticoagulants such as warfarin)

 

Symptoms

 

Symptoms may include any of the following:

  • Bleeding from mucus membranes
  • Bleeding into joints
  • Bleeding into muscles
  • Bruising easily
  • Heavy menstrual bleeding
  • Nosebleeds that do not stop easily
  • Umbilical cord bleeding after birth

 

Exams and Tests

 

Tests that may be done include:

  • Partial thromboplastin time ( PTT )
  • Plasma factor VII activity
  • Prothrombin time ( PT )
  • Mixing study

 

Treatment

 

Bleeding can be controlled by getting intravenous (IV) infusions of normal plasma, concentrates of factor VII, or genetically produced (recombinant) factor VII.

You will need frequent treatment during bleeding episodes because factor VII does not last for long inside the body. A form of factor VII called NovoSeven can also be used.

If you have factor VII deficiency due to a lack of vitamin K, you can take this vitamin 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)

 

You can expect a good outcome with proper treatment.

Inherited factor VII deficiency is a lifelong condition.

The outlook for acquired factor II 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.

 

Possible Complications

 

Complications may include:

  • Excessive bleeding (hemorrhage)
  • Stroke or other nervous system problems from central nervous system bleeding
  • Joint problems in severe cases when bleeding happens often

 

When to Contact a Medical Professional

 

Get emergency treatment right away if you have severe, unexplained bleeding.

 

Prevention

 

There is no known prevention for inherited factor VII 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

     

      Tests for Factor VII 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.

      The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

       
       
       

       

       

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