Diagnostic and management practices for inherited fibrinogen disorders: a nationwide survey of Italian Hemophilia Treatment Centers

Background: inherited fibrinogen disorders are characterized by a spectrum of quantitative or qualitative fibrinogen deficiency associated with both a hemorrhagic and thrombotic risk.Methods: a nationwide survey was conducted in 19 Italian Hemophilia Treatment Centers in order to investigate current diagnostic and management practices for inherited fibrinogen disorders (afibrinogenemia, hypofibrinogenemia, and dysfibrinogenemia).Results: the survey revealed a strong consensus (95% of centers) on the necessity of both functional and antigenic fibrinogen assays for diagnosis, and a preference for fibrinogen concentrate-based treatment (used by >85% of centers). However, significant heterogeneity was observed in critical areas: for hypofibrinogenemia, the definition of a «severe» form varied among centers, and the perceived risk of spontaneous intracranial hemorrhage remained a major point of uncertainty (up to 47% of respondents answered ‘don’t know’ for this risk in hypofibrinogenemia and dysfibrinogenemia). Furthermore, intervention thresholds for major surgical procedures showed a high degree of variability across all three disorders.Conclusions: the findings highlight areas of consistent practice alongside those requiring further standardization and collaborative research to optimize the care of individuals with these rare bleeding and thrombotic conditions.