Afibrinogenemia; Hypofibrinogenemia; Dysfibrinogenemia; Factor I deficiency
Congenital fibrinogen deficiency is a very rare, inherited blood disorder in which the blood does not clot normally. It affects a protein called fibrinogen. This protein is needed for the blood to clot.
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This disease is due to abnormal genes. Fibrinogen is affected depending on how the genes are inherited:
When the abnormal gene is passed down from both parents, a person will have a complete lack of fibrinogen (afibrinogenemia).
When the abnormal gene is passed down from one parent, a person will have either a reduced level of fibrinogen (hypofibrinogenemia) or a problem with the function of fibrinogen (dysfibrinogenemia). Sometimes, these two fibrinogen problems can occur in the same person.
People with a complete lack of fibrinogen may have any of the following bleeding symptoms:
Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.