Hemophilia B

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Blood cells	Blood clots	X-linked recessive genetic defects	X-linked recessive genetic defects - how boys are affected
X-linked recessive genetic defects - how girls are affected

Christmas disease; Factor IX hemophilia

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 regular prophylactic infusions.

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

For additional information, see hemophilia support group.

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.

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

• 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.

Review Date: 05/03/2006
Reviewed By: William Matsui, MD, Assistant Professor of Oncology, Division of Hematologic Malignancies, The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, Baltimore, MD. Review provided by VeriMed Healthcare Network.