Abstract Number: PB2503
Meeting: ISTH 2020 Congress
Background: Fibrinogen is an essential factor for pregnancy, being actually involved in trophoblastic adhesion and migration, and in placental integration. In order to fulfill its role, fibrinogen has to be in sufficient quantity and quality, with a cut-off rate estimated at 1 g/L.
Aims: The authors report the management of a pregnancy in a 26 year-old Syrian woman with an important obstetrical history owing to a hypodysfibrinogenemia. In fact, she underwent a fetal loss at 6 months, several early miscarriages, and a miscarriage at the 17th week secondary to a placental abruption associated with a severe bleeding estimated at 4000 mL. However, two full-term deliveries successfully occurred in Syria, each ended in a uterine embolization on account of a severe postpartum bleeding.
Methods: Her hypodysfibrinogenemia is characterized by a basal fibrinogen rate at 0.6 g/L and 1.2 g/L, respectively with the Clauss activity and the immunologic assays. The others hemostatic parameters are strictly normal. She carries two heterozygote mutations on the FGG gene, namely c.1086T>C on exon 8 responsible for hypofibrinogenemia, and c.124G>A on exon 3 whose impact remains unknown. The familial investigation revealed that her two children and her sister carry the same hypodysfibrinogenemia.
Results: A later pregnancy was recently managed in France. This reached successfully full term thanks to a therapeutic protocol used until the 34th week, consisting on infusions of fibrinogen concentrates two times per week, a daily injection of 4000 IU of enoxaparin, and a daily dose of 75 mg of aspirin. A caesarean section was made at the 40th week under fibrinogen concentrates in order to prevent bleedings. The postpartum follow-up was very good, without any complications.
Conclusions: This case illustrates the success of a prophylaxis regimen, associating fibrinogen concentrates and antithrombotic drugs, in a young woman with hypodysfibrinogenemia. These very rare situations should be collected in an international register.
To cite this abstract in AMA style:Sattler L, Gerout A-, Feugeas O, Herb A, Grunebaum L, Desprez D. Management of a Pregnancy in a Woman with Hypodysfibrinogenemia: An Experiment in Strasbourg [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/management-of-a-pregnancy-in-a-woman-with-hypodysfibrinogenemia-an-experiment-in-strasbourg/. Accessed November 28, 2023.
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