Dysfibrinogenemia Clinical Presentation

Updated: Apr 28, 2022
  • Author: Guy B Faguet, MD; Chief Editor: Perumal Thiagarajan, MD  more...
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Presentation

History

In congenital dysfibrinogenemia, bleeding is usually mild and may not manifest until after surgical procedures or major traumatic events. Rarely, however, patients can experience combined hemorrhagic and thrombotic tendencies. In female patients with congenital afibrinogenemia, recurrent massive intra-abdominal bleeding due to rupture of Graafian follicle during ovulation has been reported. [11] In a review of 101 patients with congenital dysfibrinogenemia, the incidence of major bleeding and thrombotic events was 19.2% and 30.1%, respectively. [10]

Patients with acquired dysfibrinogenemia often have no history of bleeding or clotting, and family history is not significant for hematologic events. [1] When present, clinical manifestations of acquired dysfibrinogenemia are heterogeneous and include major bleeding or thrombosis, chronic thromboembolic pulmonary hypertension, and renal amyloidosis. [12]

Bleeding may involve any of the following:

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

Thrombotic events that may occur include the following:

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Physical Examination

In the absence of bleeding or thrombosis. the physical examination provides no clues suggesting congenital or acquired dysfibrinogenemia.  

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