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Hematology

35+ Years Treating Hemophilia

CSL Behring has over 35 years of experience in the field of hematology. From disorders that span the coagulation cascade to the management of urgent reversal of acquired bleeding, CSL Behring continues to invest in this area and remains committed to continuing our innovations in this field.

Fibrinogen Deficiency

Fibrin strands in a blood clot

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.

Congenital Fibrinogen Deficiency Type I

Type I (quantitative abnormalities)

Afibrinogenemia:

  • Complete absence of fibrinogen
  • Increased bleeding, both spontaneous and traumatic
  • Most severe form of congenital fibrinogen deficiency
  • Accounts for 8-9% of all rare bleeding disorders

Hypofibrinogenemia:

  • Less than normal plasma fibrinogen levels
  • Bleeding largely associated with trauma or surgery (80%)
Congenital Fibrinogen Deficiency Type II

Type II (qualitative abnormalities)

Dysfibrinogenemia:

  • Normal or low-normal plasma fibrinogen levels
  • Largely asymptomatic (~50%), traumatic bleeding events

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.

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.

For U.S. Healthcare Professionals only
For U.S. Healthcare Professionals only

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