Dysfibrinogenemia Clinical Presentation

Updated: Oct 21, 2015
  • Author: Russell Burgess, MD; Chief Editor: Perumal Thiagarajan, MD  more...
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Presentation

History

Bleeding occurs in approximately 50% of patients with an inherited fibrinogen disorder. Usually the bleeding is mild and may not manifest until after a surgical procedure. Patients with severe liver disease may experience extreme bleeding. Bleeding may involve the following:

  • Postoperative bleeding
  • Postoperative wound dehiscence
  • Defective wound healing
  • Bruising
  • Severe hemorrhage (rare)
  • Mild soft-tissue hemorrhage

Thrombotic events attributable to dysfibrinogenemia occur in less than 10% of patients with hereditary dysfibrinogenemias. Thrombotic events that may occur include the following:

Combined bleeding and thrombotic tendencies are extremely rare and associated only with congenital dysfibrinogenemias.

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Physical

Although many patients with inherited dysfibrinogenemia remain asymptomatic, signs that arise tend to be associated with poor wound healing, surgical wound dehiscence, and postsurgical bleeding out of proportion to that expected.

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Causes

See the list below:

  • Congenital dysfibrinogenemias are most often inherited in an autosomal dominant or codominant fashion. Several variants are inherited autosomal recessively.
  • Acquired dysfibrinogenemias occur in severe liver disease. The fibrinogen molecule produced by the impaired liver is not functional or able to form a stable fibrin clot.
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