Learn More About Factor I Deficiency

Types

There are three different types of factor I deficiency. One type, hypofibrinogenemia, occurs when a patient’s blood level of factor I protein is lower than usual, while patients with afibrinogenemia have no factor I protein at all. People with the third type, dysfibrinogenemia, have normal levels of factor I protein, but the protein doesn’t work. This can lead to bleeding problems, clotting problems, or both. Afibrinogenemia is passed to children by genes from both parents. The other types only require genes from one parent.

Bleeding from the nose, gums, or tongue is common with all three types. Some patients bleed internally, with blood collecting in joints and around muscles. Rarely, bleeding can occur in the brain or other internal organs. Women with congenital afibrinogenemia can bleed more than usual during menstruation. They might need treatment to carry a pregnancy to term.

Afibrinogenemia affects about 150–300 people in the United States. The other two types are even more rare. Patients are usually diagnosed between birth and early childhood.


Treatment

The only FDA-approved treatment for patients with afibrinogenemia and hypofibrinogenemia is RiaSTAP® (fibrinogen concentrate [human]). Patients with dysfibrinogenemia are treated with plasma or cryoprecipitate, since RiaSTAP can cause too much clotting.

Sources

Genetics Home Reference, Food and Drug Administration, HemAware, National Hemophilia Foundation, MedScape.

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