Congenital fibrinogen 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.
Afibrinogenemia; Hypofibrinogenemia; Dysfibrinogenemia; Factor I deficiency
This disease is due to abnormal genes. Fibrinogen is affected depending on how the genes are inherited:
People with a complete lack of fibrinogen may have any of the following bleeding symptoms:
People with a reduced level of fibrinogen bleed less often and the bleeding is not as severe. Those with a problem with the function of fibrinogen often don't have symptoms.
Exams and Tests
If your health care provider suspects this problem, you will have lab tests to confirm the type and severity of the disorder.
The following treatments can be used for bleeding episodes or to prepare for surgery:
People with this condition should get the hepatitis B vaccine. Having many transfusions raises your risk of getting hepatitis.
Excessive bleeding is common with this condition. These episodes may be severe, or even fatal. Bleeding in the brain is a leading cause of death in people with this disorder.
Complications may include:
When to Contact a Medical Professional
Call your provider or seek emergency care if you have excessive bleeding.
Tell your surgeon before you have surgery if you know or suspect you have a bleeding disorder.
This is an inherited condition. There is no known prevention.
Gailani D, Wheeler AP, Neff AT. Rare coagulation factor deficiencies. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 137.
Ragni MV. Hemorrhagic disorders: coagulation factor deficiencies. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 174.
Review Date: 1/29/2019
Reviewed By: 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.
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