What causes fibrinogen disorders?

Updated: Oct 06, 2020
  • Author: Irene S Pakos, DO; Chief Editor: Perumal Thiagarajan, MD  more...
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Answer

Answer

Approximately 300 abnormal fibrinogens have been reported, and about 83 structural defects have been identified. [4] The most common structural defect involves the fibrinopeptides and their cleavage sites; the second most common involves the gamma-chain polymerization region.

Mechanisms of dysfibrinogenemias include the following:

  • Impaired release of fibrinopeptides

  • Defective fibrin polymerization

  • Abnormal cross-linking by activated factor XIIIa (factor XIIIa)

  • Abnormal interactions with platelets

  • Defective fibrinolysis

  • Defective assembly of the fibrinolytic system

  • Abnormal calcium binding

Some patients with dysfibrinogenemia have additional hemostasis defects, including factor V Leiden and deficiencies in antithrombin, protein C, and protein S. [5]


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