Congenital fibrinogen deficiency (CFD) can be characterized as either a quantitative or qualitative deficiency. Both forms of the disease impair the clotting ability of the coagulation cascade and can result in uncontrolled bleeding.

Of the three types of CFD, afibrinogenemia and hypofibrinogenemia are quantitative deficiencies, whereas dysfibrinogenemia* is a qualitative deficiency.

Types of Congenital Fibrinogen Deficiency
Type Description
Afibrinogenemia (quantitative deficiency)
  • Quantitative deficiency characterized by a complete absence or extremely low levels of fibrinogen
  • Estimated prevalence of 1 per million in general population1
  • Severe and may be life-threatening
Hypofibrinogenemia (quantitative deficiency)
  • Quantitative deficiency characterized by abnormally low fibrinogen levels
  • Number of patients affected is currently unknown
  • Bleeding episodes are usually mild

Dysfibrinogenemia (qualitative deficiency)

  • Qualitative deficiency in which individuals have normal levels of fibrinogen (200 to 450 mg/dL) but circulating fibrinogen does not function properly.
  • Patients with dysfibrinogemia are often asymptomatic
  1. Acharya SS, DiMichele DM. Rare inherited disorders of fibrinogen. Hemophilia. 2008; 14:1151-1158.

Important Safety Information

RiaSTAP®, Fibrinogen Concentrate (Human), is indicated for the treatment of acute bleeding episodes in patients with congenital fibrinogen deficiency, including afibrinogenemia and hypofibrinogenemia.

RiaSTAP is contraindicated in patients with known anaphylactic or severe systemic reactions to human plasma-derived products.

Monitor patients for early signs of anaphylaxis or hypersensitivity reactions and if necessary, discontinue administration and institute appropriate treatment. Thrombotic events have been reported in patients receiving RiaSTAP; weigh the benefits of administration versus the risks of thrombosis.

RiaSTAP is made from pooled human plasma. Products made from human plasma may contain infectious agents, eg, viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent.

The most serious adverse reactions observed are thrombotic episodes (pulmonary embolism, myocardial infarction, deep vein thrombosis) and anaphylactic reactions. The most common adverse reactions observed in clinical studies (frequency >1%) were fever and headache.

Please see full prescribing information for RiaSTAP.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch or call 1-800-FDA-1088.