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Hamostaseologie
DOI: 10.1055/a-2739-3030
Original Article

The Hypofibrinolysis State Associated with the Dysfibrinogenemia Dusart is Mainly Related to the Altered Fibrin Clot Structure

Authors

  • Rita Marchi

    1   Division of Angiology and Hemostasis, Department of Medicine, University Hospitals of Geneva, University of Geneva, Geneva, Switzerland

  • Éva Katona

    2   Division of Clinical Laboratory Science, Faculty of Medicine, University of Debrecen, Debrecen, Hungary

  • Stéphane Durual

    3   Biomaterials Laboratory, University Clinics of Dental Medicine, University of Geneva, Geneva, Switzerland

  • Emmanuel Demaistre

    4   Department of Biology and Haematology, Centre hospitalier universitaire (CHU) Dijon, Dijon, France

  • Philippe Savard

    4   Department of Biology and Haematology, Centre hospitalier universitaire (CHU) Dijon, Dijon, France

  • Alessandro Casini

    1   Division of Angiology and Hemostasis, Department of Medicine, University Hospitals of Geneva, University of Geneva, Geneva, Switzerland
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Abstract

Introduction

The fibrinogen Dusart (p.Aα573Arg > Cys) is a dysfibrinogenemia associated with an increased risk of thrombosis. The aim of this study was to identify factors that could explain the hypofibrinolytic state associated with the Dusart mutation.

Methods

The fibrin α2-antiplasmin (α2-AP) incorporation was quantified by a homemade enzyme-linked immunosorbent assay. The fibrin formation and lysis were studied by turbidity at 405 nm, and the global fibrinolytic capacity (GFC) with the Lysis timer device. The plasmin generation was assessed through an automated method. The clot growth was examined using Thrombodynamics. The clot structure was evaluated by measuring the permeation constant and scanning electron microscopy (SEM).

Results

The plasma levels of D-dimer, PAI-1, FXIII, PAP, and α2-AP were within the normal range, as was α2-AP incorporation into fibrin. By turbidity the patient's clots were almost transparent, and very resistant to fibrinolysis. The patient's GFC was 51 minutes compared with 45 minutes in control. The patient's endogenous plasmin potential and the peak plasmin were increased. The Thrombodynamics analysis revealed an increased lag time and a decreased initial velocity of patient's clot growth. The fibrin clot structure was characterized by a strong reduction in clot's flow (small pores size), and very thin fibers.

Conclusion

The patient's procoagulant phenotype appears primarily driven by the formation of abnormally compact fibrin networks, leading to impaired perfusion and resistance to fibrinolysis, independent of any imbalance in fibrinolytic activators or inhibitors. These findings highlight the critical role of the fibrin clot structure in the thrombotic risk of this variant.

Keywords

dysfibrinogenemia - thrombosis - hypofibrinolysis - plasmin generation - Thrombodynamics

Supplementary Material



Publication History

Received: 06 August 2025

Accepted: 05 November 2025

Article published online:
20 January 2026

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