Congenital afibrinogenemia

Treatment

Patients may receive the liquid portion of the blood (plasma) or a blood product containing concentrated fibrinogen (cryoprecipitate) through a vein (transfusion) to treat bleeding episodes or to prepare for surgery to treat other conditions. People with this condition should have the hepatitis B vaccine because transfusion increases the risk of hepatitis.

Outlook (Prognosis)

Excess bleeding is common with this condition. These episodes may be severe, or even fatal. Bleeding in the brain is a leading cause of death in patients with this disorder.

Possible Complications

• Bleeding from the umbilical cord
• Bleeding from the mucous membranes
• Gastrointestinal bleeding
• Intracranial bleeding
• Clotting with treatment
• Development of antibodies (inhibitors) to fibrinogen with treatment

When to Contact a Medical Professional

Call your health care provider or seek emergency care if you have excessive bleeding.

Tell your surgeon before you have surgery if you know or suspect you have a blood disorder.

References

Hoffman R, Benz E, Shattil S, Furie B, Cohen H. Hematology: Basic Principles and Practice. 4th ed. Philadelphia, Pa: Churchill Livingstone, 2004.

Asselta R, Duga S, Tenchini ML. The molecular basis of quantitative fibrinogen disorders. J Thromb Haemost. October 2006;4:2115-2129.