THE CLINICAL IMPACT OF CFD

Congenital fibrinogen deficiency (CFD) profoundly affects hemostasis, leading to a spectrum of complications from severe bleeding to paradoxical thrombosis.¹ Patients with afibrinogenemia often present early with life-threatening hemorrhage such as umbilical cord bleeding, intracranial hemorrhage, and hemarthrosis, while those with hypofibrinogenemia may remain asymptomatic or experience mild mucocutaneous bleeding.¹

Risks Associated With CFD

Despite low fibrinogen levels, CFD carries a notable thrombotic risk, including venous and arterial events, due to loss of fibrin’s antithrombin-like properties and abnormal clot architecture.¹

CFD Considerations

Surgical interventions and pregnancy require meticulous planning, as inadequate fibrinogen replacement can result in catastrophic bleeding, whereas excessive supplementation may exacerbate thrombosis.¹

These challenges underscore the need for individualized management strategies and reliable fibrinogen replacement to maintain hemostatic balance.^1,2

How CFD can present in patients

Afibrinogenemia typically manifests as hemorrhage in the neonatal period, with umbilical stump bleeding reported in approximately 85% of cases³

Individuals with hypofibrinogenemia (fibrinogen levels ≤ 150 mg/dL) are typically asymptomatic but may bleed more easily if injured or if another bleeding disorder is present⁴

Females with afibrinogenemia have reported menorrhagia, menometrorrhagia, and postpartum hemorrhage⁵

Arriving at the hospital with low fibrinogen is linked to higher transfusion needs and death rates⁶

Undiagnosed CFD can pose a significant risk for patients.²

Diagnosing CFD

Get more information about CFD, management options, and treatment advancements.

Stay informed

References

May JE, Wolberg AS, Lim MY. Disorders of fibrinogen and fibrinolysis. Hematol Oncol Clin North Am. 2021;35(6):1197-1217.
Casini A. How I treat quantitative fibrinogen disorders. Blood. 2025;145(8):801-810.
de Moerloose P, Neerman-Arbez M. Congenital fibrinogen disorders. Semin Thromb Hemost. 2009;35:356-366.
Peyvandi F. Epidemiology and treatment of congenital fibrinogen deficiency. Thromb Res. 2012;130(S2):s7-s11.
Acharya SS, DiMichele DM. Rare inherited disorders of fibrinogen. Haemophilia. 2008;14:1151-1158.
Grottke O, Mallaiah S, Karkouti K, Saner F, Haas T. Fibrinogen supplementation and its indications. Semin Thromb Hemost. 2020;46(1):38-49.