Gynecologic and obstetric complications in women with congenital fibrinogen disorders: insights from the Prospective Rare Bleeding Disorders Database

Women and girls with congenital fibrinogen deficiencies (CFDs) face higher hemorrhagic risks during their reproductive years, yet data on gynecologic and obstetric complications remain limited. We aimed to rate the prevalence of heavy menstrual bleeding and obstetric complications in women with CFDs...

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Vydané v:Research and practice in thrombosis and haemostasis Ročník 9; číslo 5; s. 102960
Hlavní autori: Mohsenian, Samin, Palla, Roberta, Menegatti, Marzia, Cairo, Andrea, Siboni, Simona Maria, Neerman-Arbez, Marguerite, Karimi, Mehran, Pargantou, Helen, Asselta, Rosanna, Mikovic, Danijela, Saracevic, Marko, Laros-van Gorkom, Britta, Jacobs, Laura, Shapiro, Amy, Williamson, Adrianna, Makris, Michael, Casini, Alessandro, Peyvandi, Flora
Médium: Journal Article
Jazyk:English
Vydavateľské údaje: United States Elsevier Inc 01.07.2025
Elsevier
Predmet:
bleeding
FGA
FGB
FGG
fibrinogen
genetic
gynecological
Hematology
hypofibrinogenemia
miscarriage
obstetrical
Original
postpartum hemorrhage
FGB
genetic
hypofibrinogenemia
postpartum hemorrhage
FGA
bleeding
fibrinogen
miscarriage
FGG
obstetrical
gynecological
ISSN:2475-0379, 2475-0379
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Shrnutí:Women and girls with congenital fibrinogen deficiencies (CFDs) face higher hemorrhagic risks during their reproductive years, yet data on gynecologic and obstetric complications remain limited. We aimed to rate the prevalence of heavy menstrual bleeding and obstetric complications in women with CFDs and compare our findings with previous reports. This study analyzed data from the Prospective Rare Bleeding Disorders Database registry, including available fibrinogen activity and antigen levels, as well as clinical phenotype and genotype (2013-2020). A total of 59 women (8 afibrinogenemic, 15 hypofibrinogenemic, and 36 dysfibrinogenemic cases) were investigated, of which 32 patients had 70 pregnancies. The prevalence of heavy menstrual bleeding was comparable between hypofibrinogenemic (27%) and dysfibrinogenemic (36%) cases, with a higher frequency in afibrinogenemic (75%) cases. The rates of postpartum hemorrhage at 36% and miscarriage at 23% were notably higher than those observed in the general population (1%-10% and 10%-20%, respectively). These complications were similarly distributed among patients with dysfibrinogenemia (35% and 37%) and hypofibrinogenemia (36% and 31%). There were only 2 (4%) bleeds during pregnancy, both occurring in dysfibrinogenemic cases. Miscarriage was also observed in 50% of the asymptomatic dysfibrinogenemic patients. No significant difference in miscarriage and postpartum hemorrhage rates was found between dysfibrinogenemic individuals with and without hotspot variants (P = .94). The high rate of obstetric complications in women with CFDs highlights the need for early diagnosis and the potential need for prophylaxis, as pregnancy may also pose risks in asymptomatic cases. Hotspot variants do not appear to increase the risk of obstetric complications. •Women and girls with CFD may face more adverse outcomes.•This study used data from the Prospective Rare Bleeding Disorders Database.•In qualitative CFDs, live birth, PPH, and miscarriage rates were 64%, 23%, and 36%.•Early diagnosis and prophylaxis before pregnancy may help prevent obstetric complications.
Bibliografia:ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
content type line 23
ISSN:2475-0379
2475-0379
DOI:10.1016/j.rpth.2025.102960