Dysfibrinogenaemia

Dysfibrinogenaemia is rare and is generally associated with a reduce plasminogen concentration.

• there is a variable increased risk of thrombosis
• the prevalence in patients with venous thromboembolism is low
• prevalence in normal population is rare

Note

Fibrinogen disorders requiring replacement therapy can be either congenital or acquired, typically involving an abnormality in the amount or function of circulating fibrinogen. Classification of these disorders is as follows:

• Afibrinogenemia: An absence of circulating fibrinogen
• Hypofibrinogenemia: Reduced levels of circulating fibrinogen (<150 mg/dL)
• Dysfibrinogenemia: Circulating fibrinogen is dysfunctional
• Hypodysfibrinogenemia: Circulating fibrinogen is reduced in quantity and is functionally abnormal

Reference

1. Simurda T et al. Congenital Afibrinogenemia and Hypofibrinogenemia: Laboratory and Genetic Testing in Rare Bleeding Disorders with Life-Threatening Clinical Manifestations and Challenging Management. Diagnostics (Basel). 2021 Nov 19;11(11)