Swiss Society of Hematology award for the best abstract presented at the Swiss Oncology Hematology Congress 2025 in the field of hemostasis, transfusion medicine, vascular, laboratory medicine, benign hematology
Enhancing hemostasis in Glanzmann thrombasthenia via protein S inhibition
R. Diab, T. Knopp, M. Fiore, S. Avoue-Celerier, L. Schacher, R. Prince-Eladnani, A. Angelillo-Scherrer
Presented by: R. Diab
Glanzmann thrombasthenia (GT) is a rare inherited platelet disorder characterized by impaired aggregation and severe bleeding. This study evaluated whether partial inhibition of protein S (PS), a natural anticoagulant, could enhance thrombin generation (TG) and improve hemostasis in GT. A single subcutaneous dose of PS–siRNA in GT (Itgb3⁻/⁻) mice significantly reduced mucocutaneous and gastrointestinal bleeding, increased thrombin generation, and decreased splenomegaly, an indirect marker of chronic bleeding. Consistent with these findings, antibody-mediated PS reduction in platelet-rich plasma from GT patients significantly increased thrombin generation. Together, these results demonstrate that partial PS inhibition rebalances hemostasis in GT and support further preclinical evaluation of PS–siRNA as a novel prophylactic therapeutic strategy.
https://www.sohc.ch/abstract/awards-2025/

