Hemophilia B

Alternative Names

Treatment

Standard treatment is infusion of factor IX concentrates to replace the defective clotting factor. The amount infused depends upon the severity of bleeding, the site of the bleeding, and the size of the patient. Hepatitis B vaccine is recommended for individuals with Hemophilia B because they are at increased risk of developing hepatitis due to exposure to blood products.

To prevent a bleeding crisis, people with hemophilia and their families can be taught to administer factor IX concentrates at home at the first signs of bleeding. People with severe forms of the disease may need ongoing, preventive infusions.

Depending on the severity of the disease, factor IX concentrate may be given prior to dental extractions and surgery to prevent bleeding.

Support Groups

For additional information, see hemophilia support group.

Outlook (Prognosis)

The outcome is usually good with treatment. Most people with hemophilia are able to lead relatively normal lives. A small percentage of people develop inhibitors of factor IX, and may die from loss of blood.

People with hemophilia B should establish an ongoing relationship with a hematologist, especially one associated with a hemophilia treatment center. The ability to have quick and easy access to medical records describing their level of Factor IX, history of transfusions (including the type and amount), any complications they've had, and the type and amount of any inhibitors can be lifesaving in the event the person with hemophilia is in an emergency situation.

Possible Complications

• Chronic joint deformities, caused by recurrent bleeding into the joint, may be managed by an orthopedic specialist.
• Intracerebral hemorrhage (such as deep intracerebral hemorrhage and lobar intracerebral hemorrhage) may also occur.
• Recurrent transfusions may expose the individual to HIV and hepatitis, (however, in 1985, blood screening procedures were improved for detecting the HIV virus).
• Thrombosis may occur following use of factor IX concentrate.

When to Contact a Medical Professional

• Call your health care provider if symptoms of a bleeding disorder develop.
• Call for an appointment with your health care provider (for screening) if a family member has been diagnosed with hemophilia B.
• Call for an appointment with your health care provider if you have hemophilia B, and you plan to have children.

References