Severe Hereditary Hypofibrinogenemia in Pregnancy: A Case Report of a Novel Obstetrical Management with Thromboelastometry Guided Fibrinogen Supplementation
Grigorios Karampas,
Konstantinos Karkalemis,
Anastasia Bagiasta,
Maria-Ekaterini Lefaki,
Dimitra Metallinou,
Chryssoula Staikou,
Zoi Iliodromiti,
Rozeta Sokou,
Kassandra Tataropoulou,
Theodora Boutsikou,
Makarios Eleftheriades,
Nikolaos Vlahos,
Panagiotis Christopoulos,
Marianna Politou
Affiliations
Grigorios Karampas
Second Department of Obstetrics and Gynaecology, “Aretaieio” University Hospital, Medical School, National and Kapodistrian University of Athens, PC 11528 Athens, Greece
Konstantinos Karkalemis
Second Department of Obstetrics and Gynaecology, “Aretaieio” University Hospital, Medical School, National and Kapodistrian University of Athens, PC 11528 Athens, Greece
Anastasia Bagiasta
Second Department of Obstetrics and Gynaecology, “Aretaieio” University Hospital, Medical School, National and Kapodistrian University of Athens, PC 11528 Athens, Greece
Maria-Ekaterini Lefaki
Laboratory of Heamatology, Blood Transfusion Unit, “Aretaieio” University Hospital, Medical School, National and Kapodistrian University of Athens, PC 11528 Athens, Greece
Dimitra Metallinou
Department of Midwifery, School of Health and Care Sciences, University of West Attica, PC 12243 Athens, Greece
Chryssoula Staikou
First Department of Anesthesiology, “Aretaieio” University Hospital, Medical School, National and Kapodistrian University of Athens, PC 11528 Athens, Greece
Zoi Iliodromiti
Neonatal Department, “Aretaieio” University Hospital, Medical School, National and Kapodistrian University of Athens, PC 11528 Athens, Greece
Rozeta Sokou
Neonatal Department, “Aretaieio” University Hospital, Medical School, National and Kapodistrian University of Athens, PC 11528 Athens, Greece
Kassandra Tataropoulou
Neonatal Intensive Care Unit, Second Department of Pediatrics, “Aglaia Kyriakou” Children’s Hospital, Medical School, National and Kapodistrian University of Athens, PC 11573 Athens, Greece
Theodora Boutsikou
Neonatal Department, “Aretaieio” University Hospital, Medical School, National and Kapodistrian University of Athens, PC 11528 Athens, Greece
Makarios Eleftheriades
Second Department of Obstetrics and Gynaecology, “Aretaieio” University Hospital, Medical School, National and Kapodistrian University of Athens, PC 11528 Athens, Greece
Nikolaos Vlahos
Second Department of Obstetrics and Gynaecology, “Aretaieio” University Hospital, Medical School, National and Kapodistrian University of Athens, PC 11528 Athens, Greece
Panagiotis Christopoulos
Second Department of Obstetrics and Gynaecology, “Aretaieio” University Hospital, Medical School, National and Kapodistrian University of Athens, PC 11528 Athens, Greece
Marianna Politou
Laboratory of Heamatology, Blood Transfusion Unit, “Aretaieio” University Hospital, Medical School, National and Kapodistrian University of Athens, PC 11528 Athens, Greece
Background and Clinical Significance: Hereditary Fibrinogen Disorders (HFDs) are a group of rare, inherited coagulation disorders with a wide spectrum of clinical presentations, ranging from asymptomatic cases to severe bleeding or thrombotic events. Among these, hereditary hypofibrinogenemia (HH) poses particular challenges in obstetric care due to its unpredictable course and limited evidence-based guidelines. Case Presentation: This case report describes the novel obstetrical management of a 37 years old multiparous woman with severe HH (SHH) guided not only by fibrinogen levels but also by rotational thromboelastometry (ROTEM®), a global test of hemostasis using specific parameters such as FIBTEM® and NATEM® assays. Despite persistent low fibrinogen levels during labor and peripartum (®-based parameters to guide clinical decisions. Conclusions: Current recommendations for managing pregnancies in women with HFDs are largely based on expert consensus and exclusively use fibrinogen levels. This case supports the use of specific assays (FIBTEM® and NATEM®) of the ROTEM® global test of hemostasis as valuable tools in the obstetric management of women with HH. The use of FIBTEM® and NATEM® assays could provide individualized perinatal care, avoiding unnecessary therapeutic interventions and aiming for optimal perinatal outcomes.
