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.

RiaSTAP is not indicated for dysfibrinogenemia.

Types of Congenital Fibrinogen Deficiency1
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 population
  • 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 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 human blood. The risk of transmission of infectious agents, including viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent and its variant (vCJD), cannot be completely eliminated.

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.

Indications

RiaSTAP is indicated for the treatment of acute bleeding episodes in patients with congenital fibrinogen deficiency, including afibrinogenemia and hypofibrinogenemia.

Please see full prescribing information for RiaSTAP.

To report SUSPECTED ADVERSE REACTIONS, contact the CSL Behring Pharmacovigilance Department at 1-866-915-6958 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.