Dysfibrinogenemia Treatment & Management

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

Approach Considerations

Treatment is not indicated in the majority of patients. When clinically significant bleeding occurs, replacement therapy is indicated. Fresh frozen plasma (FFP) or cryoprecipitate should be administered, depending on bleeding severity and product availability. 

Fibrinogen replacement therapy may prevent pregnancy complications. [9]

Venous thromboembolism secondary to congenital dysfibrinogenemia should be treated with low-molecular-weight heparin. [10] Patients with recurrent thrombotic events may require long-term anticoagulation with warfarin or subcutaneous heparin. Long-term treatment recommendations have not been established and data are lacking to support superiority of any one treatment modality. [1]

Educate patients with congenital dysfibrinogenemias that it is an inherited condition and other family members may also be affected.

 

Next:

Prevention

The obstetric complications of dysfibrinogenemia include first-trimester pregnancy loss, along with hemorrhage, placental abruption, and thrombosis. Administration of prophylactic cryoprecipitate may prevent recurrent miscarriages. Miesbach et al described the use of fibrinogen concentrates to avoid pregnancy loss in women with dysfibrinogenemia. The investigators performed a retrospective study of 4 women from the same family, each of whom had dysfibrinogenemia and a history of recurrent pregnancy loss. The patients received fibrinogen concentrates from the start of pregnancy until delivery, with 3 of the 4 women achieving delivery. [16]

Previous