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Mustafa Vakur Bor1,2

  • 1Klinisk Biokemisk Afsnit, Klinisk Diagnostisk Afdeling, Syddansk Universitetshospital, Esbjerg.

Ugeskrift for Laeger
|January 18, 2024
PubMed
Summary

Congenital fibrinogen disorders, including afibrinogenemia and dysfibrinogenemia, are rare hemostasis conditions. This review updates Danish physicians on their variable symptoms, diagnosis, and management with fibrinogen concentrate or heparin.

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Area of Science:

  • Hematology
  • Coagulation Disorders
  • Rare Diseases

Background:

  • Congenital fibrinogen disorders encompass afibrinogenemia, hypofibrinogenemia, dysfibrinogenemia, and hypodysfibrinogenemia.
  • These rare hemostasis pathologies present with variable phenotypes, ranging from asymptomatic cases to severe bleeding or thrombotic events.
  • Diagnosis is often incidental, highlighting the need for increased awareness.

Conclusions:

  • An updated understanding of congenital fibrinogen disorders is crucial for effective patient management.
  • Timely diagnosis and appropriate treatment are essential for mitigating bleeding and thrombotic risks.
  • This review serves as a resource for Danish physicians managing patients with these rare hematologic conditions.