A rare, inherited blood disorder in which the blood does not clot normally due to a lack of or a malfunction involving fibrinogen, a protein necessary for coagulation.

Causes, incidence, and risk factors

This rare disease is caused by an autosomal recessive gene (which requires inheritance of the abnormal gene from both parents). There may be either a complete lack of fibrinogen or a defect in the functioning of available fibrinogen. The condition can occur in both sexes. The main risk factor is a family history of bleeding disorders.

Signs and tests

If a bleeding disorder is suspected, laboratory tests are performed to determine the type and extent. Tests include:

Treatment

Patients may be transfused with plasma (the liquid portion of the blood) or cryoprecipitate (a blood product containing concentrated fibrinogen) to treat bleeding episodes or in preparation for surgery needed to treat other conditions. People with this condition should be immunized with the hepatitis B vaccine because of the increased risk of developing hepatitis due to transfusion.

Expectations (prognosis)

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

Complications

Calling your health care provider

Call your health care provider or seek emergency care if excessive bleeding occurs. Notify your surgeon before you have surgery if you know or suspect you have a blood disorder.

Prevention

There is no known prevention. Genetic counseling may be helpful for couples in which at least one partner has this condition considering childbearing.