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Congenital dysfibrinogenemia

Key sources
The following summarized guidelines for the evaluation and management of congenital dysfibrinogenemia are prepared by our editorial team based on guidelines from the UK Haemophilia Centre Doctors Organization (UKHCDO/RCOG 2017) and the British Committee for Standards In Haematology (BCSH 2014).
1
2

Guidelines

1.Screening and diagnosis

Diagnosis
Recognize that fibrinogen disorders can be autosomal recessive (afibrinogenemia) or autosomal dominant (with quantitative and/or qualitative defects) and are associated with a variable clinical phenotype.
B
Recognize that severe fibrinogen deficiency may be associated with bleeding, but quantitative and qualitative deficiency can also be associated with thrombosis and pregnancy loss.
B
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2.Medical management

Management of bleeding
Consider administering tranexamic acid 15-20 mg/kg or 1 g QID for mild bleeding in patients with afibrinogenemia, hypofibrinogenemia, or hemorrhagic dysfibrinogenemia.
C
Consider administering fibrinogen concentrate 50-100 mg/kg, with smaller doses repeated if necessary at 2-4-day intervals to maintain fibrinogen activity > 1.0 g/L, for severe bleeding in patients with afibrinogenemia, hypofibrinogenemia, or hemorrhagic dysfibrinogenemia.
C

3.Perioperative care

Perioperative bleeding prophylaxis
Consider administering tranexamic acid 15-20 mg/kg or 1 g QID for minor surgery in patients with afibrinogenemia, hypofibrinogenemia, or hemorrhagic dysfibrinogenemia.
C
Consider administering fibrinogen concentrate 50-100 mg/kg, with smaller doses repeated if necessary at 2-4-day intervals to maintain fibrinogen activity > 1.0 g/L, for major surgery in patients with afibrinogenemia, hypofibrinogenemia, or hemorrhagic dysfibrinogenemia.
C

More topics in this section

  • Perioperative thromboprophylaxis

4.Specific circumstances

Pregnant patients, general principles
Elicit a personal and/or family history to guide management decisions, as hypofibrinogenemia and dysfibrinogenemia are associated with variable clinical phenotypes.
B
Offer expectant management in pregnant patients or neonates if there is no personal or family history of bleeding or thrombosis.
B

More topics in this section

  • Pregnant patients (bleeding prophylaxis)

  • Pregnant patients (thromboprophylaxis)

  • Pregnant patients (interventions to avoid)

5.Preventative measures

Long-term bleeding prophylaxis
Consider initiating long-term prophylaxis with fibrinogen concentrate, initially 50-100 mg/kg once weekly and then adjusted to maintain trough fibrinogen activity > 0.5 g/L, in patients with a personal or family history of severe bleeding or with fibrinogen activity < 0.1 g/L.
C
Consider administering pathogen-reduced cryoprecipitate 15-20 mL/kg if fibrinogen concentrate is not available.
C