Other Disorders

Factor Deficiency Incidence Common Symptoms
Factor I (Fibrinogen) 1 in 1,000,000

Afibrinogenemia is the complete absence of fibrinogen. Hypofibrinogenemia is a low level of fibrinogen—less than 100mg in 1dL of blood. Both conditions are inherited in an autosomal fashion and can affect males and females.

The severity of the disorder is related to the amount of fibrinogen. Afibrinogenemia is usually discovered in newborns and can cause bleeding from the umbilical cord, genitourinary tract, or central nervous system. People with hypofibrinogenemia may have little, moderate, or severe bleeding.

Factor II (Prothrombin) 1 in 1,000,000

Prothrombin is a vitamin K-dependent proenzyme that functions in coagulation. There are two types of this deficiency, a congenital version called hypoprothrombinemia, and an acquired version called dyspothrombinemia.

A life-long bleeding disorder when congenital, it's extremely rare. In fact, only 30 cases of this hereditary clotting factor defect have been identified in the whole world.

Acquired factor II deficiency is more common. It results from vitamin K deficiency, severe liver disease, and therapeutic use of anticoagulant drugs. Risk factors for vitamin K deficiency are prolonged use of antibiotics, bile duct obstruction, and intestinal malabsorption (inadequate absorption of nutrients from the intestinal tract) disorders. Some newborns are born with vitamin K deficiency.

If it is caused by liver disease, the outcome depends on the control of the liver problem. Vitamin K administration will correct vitamin K deficiency.

Either form may lead to severe bruising, excessive menstrual bleeding, postoperative hemorrhage, or occasionally muscle bleeds.

A congenital factor II deficiency is a very rare inherited disorder that results in deficient blood clotting. The disorder is not sex-linked as is hemophilia. It affects both males and females with equal frequency. It is autosomal recessive, which means if the clotting defect is inherited from a parent, the child will be a genetic carrier of the condition, but may or may not have symptoms. A family history of bleeding disorder is a risk factor.

Factor V (Labile Factor) Less than 1 in 1,000,000

This deficit was identified in Norway in 1943. Since then about 150 cases have been reported, occurring in both men and women. The exact frequency of this rare disorder is unknown, but is estimated to be one per million.

The role of factor V is to accelerate the activity of thrombin. When levels of factor V are low, blood clotting is delayed or it progresses slowly. People with this deficiency may have occasional nosebleeds, excessive menstrual bleeding, and bruising, although many have no symptoms. The first sign of this condition may be bleeding following surgery.

In this disorder, bleeding ranges from mild to severe. The disease is similar to hemophilia, except bleeding into joints is uncommon. Bleeding can occur almost anywhere in the body, and death from hemorrhage has occurred with this disorder. Excessive bleeding with menstrual periods and postpartum hemorrhage occurs frequently.

A family history of a bleeding disorder is a risk factor. Men and women are affected equally.

The probable outcome is good with proper diagnosis and treatment.

Factor VII (Stable Factor) 1 in 500,000

This extremely rare disorder can be inherited or acquired by persons without hemophilia who take Coumadin, a drug used to inhibit blood clotting.

In this disorder, bleeding can vary from mild to severe within the same person over time. Bleeding doesn't always correspond with the severity of the deficiency shown in blood tests. A history of bleeding may occur in infancy or childhood.

Gastrointestinal and central nervous system bleeding can also occur. This disorder occurs in one in 500,000 males and females. Congenital factor VII deficiency should be distinguished from acquired factor VII deficiency that may result from liver disease, vitamin K deficiency, or other malabsorption conditions.

When levels of the factor are less than 1% of normal, bleeding can be severe. The trauma of birth may cause bleeding in the head of a newborn. Circumcision may cause prolonged bleeding. Children and adults may suffer bleeding from nose, gums, or gastrointestinal tract, and women may suffer excessive menstrual bleeding. The probable outcome is good with proper treatment.

Factor Deficiency

Factor I (Fibrinogen)

Incidence

1 in 1,000,000

Common Symptoms

Afibrinogenemia is the complete absence of fibrinogen. Hypofibrinogenemia is a low level of fibrinogen—less than 100mg in 1dL of blood. Both conditions are inherited in an autosomal fashion and can affect males and females. The severity of the disorder is related to the amount of fibrinogen. Afibrinogenemia is usually discovered in newborns and can cause bleeding from the umbilical cord, genitourinary tract, or central nervous system. People with hypofibrinogenemia may have little, moderate, or severe bleeding.

Factor Deficiency

Factor II (Prothrombin)

Incidence

1 in 1,000,000

Common Symptoms

Prothrombin is a vitamin K-dependent proenzyme that functions in coagulation. There are two types of this deficiency, a congenital version called hypoprothrombinemia, and an acquired version called dyspothrombinemia. A life-long bleeding disorder when congenital, it's extremely rare. In fact, only 30 cases of this hereditary clotting factor defect have been identified in the whole world. Acquired factor II deficiency is more common. It results from vitamin K deficiency, severe liver disease, and therapeutic use of anticoagulant drugs. Risk factors for vitamin K deficiency are prolonged use of antibiotics, bile duct obstruction, and intestinal malabsorption (inadequate absorption of nutrients from the intestinal tract) disorders. Some newborns are born with vitamin K deficiency. If it is caused by liver disease, the outcome depends on the control of the liver problem. Vitamin K administration will correct vitamin K deficiency. Either form may lead to severe bruising, excessive menstrual bleeding, postoperative hemorrhage, or occasionally muscle bleeds. A congenital factor II deficiency is a very rare inherited disorder that results in deficient blood clotting. The disorder is not sex-linked as is hemophilia. It affects both males and females with equal frequency. It is autosomal recessive, which means if the clotting defect is inherited from a parent, the child will be a genetic carrier of the condition, but may or may not have symptoms. A family history of bleeding disorder is a risk factor.

Factor Deficiency

Factor V (Labile Factor)

Incidence

Less than 1 in 1,000,000

Common Symptoms

This deficit was identified in Norway in 1943. Since then about 150 cases have been reported, occurring in both men and women. The exact frequency of this rare disorder is unknown, but is estimated to be one per million. The role of factor V is to accelerate the activity of thrombin. When levels of factor V are low, blood clotting is delayed or it progresses slowly. People with this deficiency may have occasional nosebleeds, excessive menstrual bleeding, and bruising, although many have no symptoms. The first sign of this condition may be bleeding following surgery. In this disorder, bleeding ranges from mild to severe. The disease is similar to hemophilia, except bleeding into joints is uncommon. Bleeding can occur almost anywhere in the body, and death from hemorrhage has occurred with this disorder. Excessive bleeding with menstrual periods and postpartum hemorrhage occurs frequently. A family history of a bleeding disorder is a risk factor. Men and women are affected equally. The probable outcome is good with proper diagnosis and treatment.

Factor Deficiency

Factor VII (Stable Factor)

Incidence

1 in 500,000

Common Symptoms

This extremely rare disorder can be inherited or acquired by persons without hemophilia who take Coumadin, a drug used to inhibit blood clotting. In this disorder, bleeding can vary from mild to severe within the same person over time. Bleeding doesn't always correspond with the severity of the deficiency shown in blood tests. A history of bleeding may occur in infancy or childhood. Gastrointestinal and central nervous system bleeding can also occur. This disorder occurs in one in 500,000 males and females. Congenital factor VII deficiency should be distinguished from acquired factor VII deficiency that may result from liver disease, vitamin K deficiency, or other malabsorption conditions. When levels of the factor are less than 1% of normal, bleeding can be severe. The trauma of birth may cause bleeding in the head of a newborn. Circumcision may cause prolonged bleeding. Children and adults may suffer bleeding from nose, gums, or gastrointestinal tract, and women may suffer excessive menstrual bleeding. The probable outcome is good with proper treatment.