Factor V deficiency

Factor V deficiency

Definition:

Factor V deficiency is an inherited abnormal blood coagulation disorder caused by a deficiency of the plasma protein Factor V.

Alternative Names:

Parahemophilia; Owren's disease

Causes, incidence, and risk factors:

Normal blood coagulation is a complex process involving as many as 20 different plasma proteins, which are known as blood coagulation factors. A series of complex chemical reactions using these factors takes place very rapidly to form an insoluble protein called fibrin that stops bleeding.

When certain coagulation factors are deficient or missing, the chain reaction does not take place normally. Factor V deficiency is caused by inheriting a defective Factor V gene. In this disorder, bleeding ranges from mild to severe.

The disease is similar to hemophilia, except bleeding into joints is less common. Bleeding can occur almost anywhere in the body, and death from hemorrhage has occurred with this disorder.

Excessive bleeding with menstrual periods and after delivery occurs frequently. A family history of a bleeding disorder is a risk factor. Men and women are affected equally, and the incidence is about 1 in 1 million.

Symptoms:

Bleeding into the skin
Excessive bruising
Nose bleeds
Bleeding of the gums
Excessive menstrual bleeding
Prolonged or excessive loss of blood with surgery or trauma
Umbilical stump bleeding

Signs and tests:

Factor V assay showing decreased activity
Slightly prolonged bleeding time (in some people)
Prolonged partial thromboplastin time
Prolonged prothrombin time
Normal thrombin time

Treatment:

Fresh plasma or fresh frozen plasma infusions will correct the deficiency temporarily and should be given daily during a bleeding episode or after surgery.

Support Groups:

The stress of illness can often be helped by joining a support group where members share common experiences and problems. See hemophilia - support group.

Expectations (prognosis):

The probable outcome is good with diagnosis and proper treatment.

Complications:

Severe hemorrhage could occur.

Calling your health care provider:

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

Prevention:

This is an inherited disorder; there is no known prevention.

Review Date: 5/6/2002
Reviewed By: Rebecca Elstrom, M.D., Division of Hematology-Oncology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network (10/14/2001).

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