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Factor V deficiency

Parahemophilia; Owren disease; Bleeding disorder - Factor V deficiency

 

Factor V deficiency is a condition that is passed down through families, which affects the ability of the blood to clot.

Causes

 

Blood clotting is a complex process involving as many as 20 different proteins in blood plasma. These proteins are called blood coagulation factors.

Factor V deficiency is caused by a lack of Factor V. When certain blood clotting factors are low or missing, your blood does not clot properly.

Factor V deficiency is rare. It may be caused by:

  • A defective Factor V gene passed down through families (inherited)
  • An antibody that interferes with normal Factor V function

You can get an antibody that interferes with Factor V:

  • After giving birth
  • After being treated with a certain type of fibrin glue
  • After surgery
  • With autoimmune diseases and certain cancers

Sometimes the cause is unknown.

The disease is similar to hemophilia, except bleeding into joints is less common. In the inherited form of Factor V deficiency, a family history of a bleeding disorder is a risk factor.

 

Symptoms

 

Excessive bleeding with menstrual periods and after childbirth often occurs. Other symptoms can include:

  • Bleeding into the skin
  • Bleeding of the gums
  • Excessive bruising
  • Nosebleeds
  • Prolonged or excessive loss of blood with surgery or trauma
  • Umbilical stump bleeding

 

Exams and Tests

 

Tests to detect Factor V deficiency include:

  • Factor V assay
  • Blood clotting tests, including partial thromboplastin time (PTT) and prothrombin time
  • Bleeding time

 

Treatment

 

You will be given fresh blood plasma or fresh frozen plasma infusions during a bleeding episode or after surgery. These treatments will correct the deficiency temporarily.

 

Outlook (Prognosis)

 

The outlook is good with diagnosis and proper treatment.

 

Possible Complications

 

Severe bleeding (hemorrhage) could occur.

 

When to Contact a Medical Professional

 

Go to the emergency room or call the local emergency number (such as 911) if you have an unexplained or prolonged loss of blood.

 

 

References

Gailani D, Neff AT. Rare coagulation factor deficiencies. In: Hoffman R, Benz EJ Jr, Silberstein LE, Heslop HE, Weitz JI, Anastasi J, eds. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, PA: Elsevier Saunders; 2013:chap 139.

Ragni MV. Hemorrhagic disorders In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 174.

Scott JP, Flood VH. Hereditary clotting factor deficiencies (bleeding disorders). In: Kliegman RM, Stanton BF, St Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016:chap 476.

 
  • Blood clot formation

    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

    Blood clots - illustration

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

    Blood clots

    illustration

    • Blood clot formation

      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

      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 V deficiency

         

           

          Review Date: 2/1/2016

          Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. 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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