Original Articles
Vol. 4 No. 3 (2025)
https://doi.org/10.4081/btvb.2025.372

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

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Published: 2 December 2025
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Nationwide survey, afibrinogenemia, hypofibrinogenemia, dysfibrinogenemia

Authors

Renato Marino
remarino64@gmail.com
https://orcid.org/0000-0003-4764-8974
Hemophilia and Thrombosis Center, University Hospital Policlinico, Bari, Italy.
Silvia Linari
Center for Bleeding Disorders and Coagulation, Department of Heart, Lung and Vessels, Careggi University Hospital, Florence, Italy.
Marzia Leotta
Hemophilia, Hemostasis and Thrombosis Unit, Department of Hemato-Oncology, Dulbecco University Hospital, Catanzaro, Italy.
Antonio Coppola
Regional Reference Center for Inherited Bleeding Disorders, University Hospital of Parma, Italy.
Chiara Biasoli
Hemophilia Center, "Bufalini" Hospital, Cesena, Italy.
Maria Elisa Mancuso
Center for Thrombosis and Hemorrhagic Diseases, IRCCS Humanitas Research Hospital, Rozzano (MI); Department of Biomedical Sciences, Humanitas University, Pieve Emanuele (MI), Italy.
Flora Peyvandi
Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Milan, Italy.
Cristina Santoro
Hematology Unit, Policlinico Umberto I University Hospital, Rome, Italy.
Ezio Zanon
Department of Medicine, Hemophilia Center, University Hospital of Padua, Italy.
Rita Carlotta Santoro
Hemophilia, Hemostasis and Thrombosis Unit, Department of Hemato-Oncology, Dulbecco University Hospital, Catanzaro, Italy.

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.

 

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How to Cite



1.
Marino R, Linari S, Leotta M, Coppola A, Biasoli C, Mancuso ME, et al. Diagnostic and management practices for inherited fibrinogen disorders: a nationwide survey of Italian Hemophilia Treatment Centers. Bleeding Thromb Vascul Biol [Internet]. 2025 Dec. 2 [cited 2026 Feb. 23];4(3). Available from: https://www.btvb.org/btvb/article/view/372
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