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.
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 clots (fibrin clots) are the clumps that result when blood coagulates.
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:
- 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 911 or the local emergency number 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. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 165.
Scott JP, Flood VH. Hereditary clotting factor deficiencies (bleeding disorders). In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 503.
Version Info
Last reviewed on: 1/25/2022
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, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
