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Factor V deficiency

Parahemophilia; Owren disease; Bleeding disorder - factor V deficiency

Factor V deficiency is a bleeding disorder that is passed down through families. It 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 develop 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:

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.

Support Groups

The following resources can provide more information on factor V deficiency:

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, Wheeler AP, Neff AT. Rare coagulation factor deficiencies. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 137.

Ragni MV. Hemorrhagic disorders: coagulation factor deficiencies. In: Goldman L, Schafer AI, eds. Goldman-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

      Review Date: 1/19/2018

      Reviewed By: Richard LoCicero, MD, private practice specializing in Hematology and Medical Oncology, Longstreet Cancer Center, Gainesville, GA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, 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., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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