Fibrinogen Deficiency

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fibrinogen overview 2 Fibrinogen deficiency is caused by an absence/ low levels of fibrinogen or dysfunctional fibrinogen.

May lead to bleeding or clotting events

Fibrinogen deficiency can be inherited (congenital) or acquired.

Two types of congenital fibrinogen deficiency:

Type I deficiencies (quantitative abnormalities): Afibrinogenemia or Hypofibrinogenemia
Type II deficiencies (qualitative abnormalities): Dysfibrinogenemia

Congenital fibrinogen deficiencies affect males and females equally.

Bleeding manifestations in afibrinogenemia can range from mild to severe and can include:

Nosebleeds
Oral bleeding
Joint and muscle bleeding
Gastrointestinal bleeding
Heavy menstrual bleeding
Traumatic and surgical bleeding

In hypofibrinogenemia, bleeding episodes are usually mild; bleeding generally occurs following trauma or surgery.

Patients with congenital fibrinogen deficiency experiencing bleeding utilize fibrinogen replacement therapy to increase plasma fibrinogen levels.

Options for treatment include cryoprecipitate, fresh frozen plasma and fibrinogen concentrate.

Reference

1. Asselta R, Duga S, Tenchini M. Molecular basis of quantitative fibrinogen disorders. J Thromb Haemost. 2006;4(10):2115-2129. 2. Peyvandi F, Haertel S, Knaub S, Mannucci PM. Incidence of bleeding symptoms in 100 patients with inherited afibrinogenemia or hypfibrinogenemia. J Thromb Haemost. 2006;4(7):1634-1637. 3. Bevan et al. Fibrinogen replacement therapy. Thromb Res. 2009;124 Suppl 2:S12-516.