Congenital afibrinogenemia is a rare, inherited blood disorder in which the blood does not clot normally. It occurs when there is a complete lack (deficiency) of a protein called fibrinogen, which is needed for the blood to clot.
Causes, incidence, and risk factors
This rare disease is caused by an abnormal gene that must be passed down from both parents. It causes a severe lack of fibrinogen.
Milder forms can occur in people who inherit only one abnormal gene from their parents. (Dysfibrinogenemia, in which there is a defect in fibrinogen function, is a different condition.)
Congenital afibrinogenemia can occur in males or females. The main risk factor is a family history of bleeding disorders.
David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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