Factor V deficiency
Overview
Alternative Names: Parahemophilia; Owren's disease Factor V deficiency is an inherited condition that affects the ability of the blood to clot.
Symptoms
* Bleeding into the skin * Bleeding of the gums * Excessive bruising * Excessive menstrual bleeding * Nosebleeds * Prolonged or excessive loss of blood with surgery or trauma * Umbilical stump bleeding
Causes
Normal blood coagulation is a complex process involving as many as 20 different proteins in blood plasma, which are known as blood coagulation factors. A series of complex chemical reactions using these factors takes place very rapidly to form a protein called fibrin, which stops bleeding. Factor V deficiency is caused by a lack of the plasma protein Factor V. When certain coagulation factors are low or missing, the chain reaction does not take place normally. Factor V is rare, and can be caused by inheriting a defective Factor V gene or by acquiring an antibody that interferes with normal Factor V function. You can acquire an inhibitor of 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. Excessive bleeding with menstrual periods and after delivery often occurs. Men and women are affected equally. About 1 person per 1 million has the disorder.
Prevention
This is an inherited disorder; there is no known prevention.
Diagnosis
* Factor V assay showing decreased activity * Normal thrombin time * Prolonged partial thromboplastin time * Prolonged prothrombin time * Slightly prolonged bleeding time (in some people)
Prognosis
The outlook is good with diagnosis and proper treatment.
Treatment
You should receive fresh blood plasma or fresh frozen plasma infusions during a bleeding episode or after surgery. These treatments will correct the deficiency temporarily.
Resources
This is an inherited disorder; there is no known prevention.