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Therapeutic Areas and Products

Hematology

Fibrinogen Deficiency

Overview

Fibrin strands in a blood clot

Fibrinogen deficiency: 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.

Types of Congenital Fibrinogen Deficiency

                                                  Type I (quantitative abnormalities) Type II (qualitative abnormalities)
Afibrinogenemia Hypofibrinogenemia Dysfibrinogenemia
Complete absence of fibrinogen Less than normal plasma fibrinogen levels Normal or low-normal plasma finbrinogen levels
Increased bleeding, both spontaneous and traumatic Bleeding largely associated with trauma or surgery (80%) Largely asymptomatic (~50%), traumatic bleeding events
Most severe form of congenital fibrinogen deficiency
Accounts for 8-9% of all rare bleeding disorders

Clinical Presentation

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.

Management

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.

Cryoprecipitate Fresh frozen plasma Fibrinogen concentrate
Standardised fibrinogen content No No Yes
Volume to be infused Medium Large Small
Undergoes virus inactivation, eg. pasteurisation Not Always Not Always Yes
Thawing (leading to a delay in time to infusion) Yes Yes No
Blood matching Yes Yes No

1. Asselta R, Duga S, Tenchini M. Molecular basis of quantitative fibrinogen disorders. J Thromb Haemost. 2006;4(10):2115-2129. 2. Figure source: https://ib.bioninja.com.au/standard-level/topic-6-human-physiology/63-defence-against-infectio/clotting.html 3. 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. 4. Bevan et al. Fibrinogen replacement therapy. Thromb Res. 2009;124 Suppl 2:S12-516.

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