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About Congenital Fibrinogen Deficiency (CFD)

Congenital fibrinogen deficiency (CFD) is a very rare, inherited bleeding disorder in which the body’s ability to form blood clots is impaired. Depending on the severity of the deficiency, patients might be asymptomatic or might experience uncontrolled bleeding—either spontaneously or in response to surgery or other trauma.

Fibrinogen deficiency can be characterized as a quantitative or qualitative deficiency.* Individuals with a quantitative fibrinogen deficiency have abnormally low levels of fibrinogen (<200 to 450 mg/dL) circulating in their bloodstream.

A qualitative fibrinogen deficiency occurs when there is a structural defect in the fibrinogen molecule that results in functional impairment. Individuals with this type of deficiency usually have normal plasma levels of fibrinogen proteins, but those proteins do not function properly.

CFD is caused by a mutation in one of the three genes responsible for producing fibrinogen. Because CFD is not a sex-linked genetic disorder (ie, it is autosomal), it is equally likely to occur in males and females.

* RiaSTAP^® is not indicated for a qualitative fibrinogen deficiency.

History

It wasn’t until 1920 that CFD came to the attention of the medical world. Two German physicians, Fritz Rabe and Eugene Salomon, tested a 9-year-old boy with lifelong unexplained bleeding problems and found a lack of fibrinogen in his blood. Nearly a century later, available treatment in the United States was still limited to fresh frozen plasma, 24-hour plasma, or cryoprecipitate. With all of these products, the exact amount of fibrinogen is unknown. Not until 2009 was a fibrinogen concentrate approved for use in the US market.

Next: The Coagulation Cascade

Learn more about Fibrinogen’s Role in Coagulation.

Learn more About RiaSTAP^®, the first and only fibrinogen concentrate for the treatment of CFD.

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 not indicated for dysfibrinogenemia.

RiaSTAP^® was approved using maximum clot firmness (MCF) as a surrogate marker likely to predict clinical benefit. Thus, the hemostatic efficacy of RiaSTAP^® in acute bleeding episodes has not been established. A post-marketing study is being conducted to verify clinical endpoints.

RiaSTAP^® is contraindicated in individuals who have manifested severe immediate hypersensitivity reactions, including anaphylaxis to RiaSTAP^® or its components.

Monitor patients for early signs of allergic 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, e.g., viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent.

The most serious adverse reactions that have been reported in subjects in clinical studies who received RiaSTAP^® are thromboembolic episodes, including myocardial infarction and pulmonary embolism, and allergic-anaphylactic reactions. The most common adverse reactions observed are allergic reactions, including chills, fever, nausea, and vomiting. Monitor patients for early signs of allergic or hypersensitivity reactions, and if necessary, discontinue administration.

Please see full Prescribing Information.

RiaSTAP^® is a trademark of CSL Behring GmbH

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