Congenital Fibrinogen (Factor I) Deficiency

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Fibrinogen (factor I) is a plasma protein that plays a critical role in the blood coagulation process. Congenital fibrinogen deficiency describes a range of inherited conditions characterized by either absence of fibrinogen, very low levels of fibrinogen or dysfunctional fibrinogen. These may result in hemorrhagic events (e.g. surgery/trauma-associated bleeding and, less commonly, spontaneous bleeding) or, occasionally, thrombotic events.

Table 1. Types of congenital fibrinogen (factor I) deficiency. 7-11
Type I deficiencies
(quantitative abnormalities)
Type II deficiencies
(qualitative abnormalities)
• The most severe form of congenital fibrinogen deficiency
• Affects 1 to 2 in a million people*
• Thought to be caused by mutations of the 3 fibrinogen genes, which causes defective fibrinogen synthesis
• Usually autosomal recessive
• Fibrinogen levels are typically <0.2 g/l
• Associated with increased bleeding
• Occasionally associated with thrombotic complications
• Symptoms are usually milder than those observed in afibrinogenemia
• Bleeding is mainly associated with trauma or surgery (80%) and less so with spontaneous events (20%)
• Usually autosomal recessive
Defined as fibrinogen levels
<1.5 g/l
• Rare – affecting 1 in a million people
• Usually autosomal dominant
• Caused by mutations in any of the 3 fibrinogen genes, which causes functional abnormalities of fibrinogen
• Fibrinogen levels are normal or low-normal
• Spontaneous bleeding is rare
• Trauma and surgery-related excessive hemorrhage can occur
• Around half of all cases are asymptomatic, 25% cause bleeding episodes and 25% cause thrombosis
*In western countries.

CSL Behring products used in the treatment of congenital fibrinogen (factor I) deficiency include: 

Product Websites*

RiaSTAP® (United States)

*Not all products are available in every country. To learn what products are available in your country, visit your CSL Behring country site.

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