PB0197 - Gynecological and obstetrical complications in women with congenital fibrinogen deficiencies

Background: Women with congenital fibrinogen disorders (CFD) are more likely to experience bleeding throughout their lives due to natural hemostatic challenges during menstruation, pregnancy, and childbirth. Nevertheless, there are currently insufficient studies addressing gynecological problems in women with CFD.

Aims: We aimed to determine the gynecological and obstetrical conditions in women with CFD.

Methods: Data were collected from 36 centers in 16 countries from 2013 to 2020 using the PRO-RBDD (Prospective Rare Bleeding Disorders Database). Clinical data, routine and specific coagulation assays including fibrinogen activity (Fg:C) and its antigen (Fg:Ag) were recorded for patients and they were classified based on the Fg:C/Fg:Ag ratio.

Results: Of 123 well-characterized CFD cases, 57 (46%) were adult women. Thirty-two (56%) of them had 68 pregnancies (1 afibrinogenemia, 16 hypofibrinogenemia, 49 dysfibrinogenemia and 2 hypodysfibrinogenemia) resulting in 47 (69%) live births (1 afibrinogenemia, 13 hypofibrinogenemia, 31 dysfibrinogenemia and 2 hypodysfibrinogenemia). There was a high rate of miscarriage (n=21, 31%), including 18 (86%) in dysfibrinogenemia and 3 (14%) in hypofibrinogenemia; 86% of miscarriages occurred in the first-trimester of gestation. Obstetrical complications including miscarriage and post-partum hemorrhage were mostly observed in dysfibrinogenemia (37%, 14%) and hypofibrinogenemia (19%, 19%) respectively (Figure 1). Women of fertile age also experienced menorrhagia in equal proportions in different groups of CFD. Two pregnancies in women with dysfibrinogenemia and hypofibrinogenemia ended in miscarriage despite receiving prophylaxis. Bleeding complication during pregnancies was observed in 2 pregnancies of dysfibrinogenemia (4%). No thrombosis events were observed following pregnancy in our cohort.

Conclusion(s): This multicentre study provided an epidemiological picture of obstetric and gynecologic complications in women with CFD. Our findings indicate that women with dysfibrinogenemia experienced more obstetrical complications, particularly miscarriage.