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Factor II Deficiency

What Is It?

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, factor II deficiency is 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 the deficiency is caused by liver disease, the outcome depends on 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 and occasional muscle bleeds.

Inheritance Pattern
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 that 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.

Symptoms and Diagnosis
Signs and symptoms vary with the level of prothrombin. Patients with levels greater than 50% of normal have no bleeding problems, whereas people with levels from 2% to 50% may easily bruise or suffer from epistaxis, menorrhagia, muscle hemorrhages, postpartum hemorrhages and hemorrhage following surgery and trauma.

Symptoms include:
• umbilical cord bleeding at birth
• nose bleeds
• abnormal menstrual bleeding
• abnormal bleeding after delivery
• bleeding after trauma
• bleeding after surgery
• easy bruising

Signs and Tests:
• prolonged prothrombin time
• prolonged partial thromboplastin time
• factor II assay showing decreased activity
• levels of prothrombin ranging from 2% to 50% of normal

Treatment depends on the severity of the disorder and the type of bleeding. Mild cases may be treated with plasma infusion. Loss of blood can be controlled by infusions of fresh frozen plasma. Severe factor II deficiencies may be treated with prothrombin complex concentrates (PCCs). When necessary, it can be treated with plasma replacement therapy. Effective control of a hemorrhage is achieved when prothrombin levels are at 30% to 50% of normal.

Genetic counseling may be helpful in the case of congenital disorders. The use of vitamin K in malabsorption and long-term antibiotic use may be preventative.

If the disorder is caused by vitamin K deficiency, then vitamin K is prescribed. Diagnosis of a bleeding disorder is important so that precautionary measures can be taken if surgery is needed or anticipated.

The National Hemophilia Foundation's Medical and Scientific Advisory Council (MASAC) made recommendations for treatment of factor II deficiency in November of 1999. They include:
• Prothrombin complex concentrates (PCCs) can be used, but these products vary considerably in the amount of factors they contain.
• Fresh frozen plasma can be used as along as it is processed to reduce the risk of viral infection.

Bleeding has to be controlled in instances of trauma or surgery, or else bleeding into the brain or skull can occur.

The information contained on the NHF web site is provided for your general information only. NHF does not give medical advice or engage in the practice of medicine. NHF under no circumstances recommends particular treatment for specific individuals and in all cases recommends that you consult your physician or local treatment center before pursuing any course of treatment.
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