Management of congenital dysfibrinogenemia in pregnancy: A challenging patient case

Research and Practice in Thrombosis and Haemostasis

Meredith Manire¹  Matthew Clarkson²  Rama Bhagavatula³  Megan Langer³  Deep Shah³  Yazan Samhouri³ 

[1] Department of Obstetrics and Gynecology West Penn HospitalAllegheny Health Network Pittsburgh Pennsylvania USA;Department of Pharmacy West Penn HospitalAllegheny Health Network Pittsburgh Pennsylvania USA;Division of Hematology and Cellular Therapy Allegheny Health Network Cancer Institute Pittsburgh Pennsylvania USA;

关键词: afibrinogenemia;    congenital;    dysfibrinogenemia;    fibrinogen;    hemorrhage;    pregnancy;   

DOI  :  10.1002/rth2.12619

来源: DOAJ

【 摘 要 】

Abstract Afibrinogenemia and congenital dysfibrinogenemia (CD) are rare conditions with limited information available for appropriate management. Previous case reports have demonstrated the safe and efficacious use of fibrinogen replacement therapy (FRT) as a therapeutic approach to prevent hemorrhage and fetal loss in pregnant women with CD. In this case report, we present a 28‐year‐old pregnant woman who sought testing for CD given her family history. She denied any current or previous bleeding symptoms. Laboratory testing confirmed the diagnosis of CD. She was treated with FRT and prophylactic anticoagulation starting in her third trimester. She had preterm labor that prompted an urgent cesarean section with FRT support. This case adds to the sparse literature about fibrinogen disorders in pregnancy, and highlights the benefits, safety, and tolerability of FRT and prophylactic anticoagulation in pregnant women with CD. Finally, it emphasizes the importance of a multidisciplinary team approach for an uneventful delivery.

【 授权许可】

Unknown