Severe Hereditary Hypofibrinogenemia in Pregnancy: A Case Report of a Novel Obstetrical Management with Thromboelastometry Guided Fibrinogen Supplementation

Abstract

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 (<100 mg/dL), favorable maternal and neonatal outcomes were achieved by relying on ROTEM^®-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.

Keywords: FIBTEM®; NATEM®; ROTEM®; hereditary hypofibrinogenemia; pregnancy.

Figure 1

Hemostasis cascade highlighting (red) impaired clot formation due to low fibrinogen levels in cases of hereditary hypofibrinogenemia.