Abstract Number: PB1486
Meeting: ISTH 2020 Congress
Background: Inherited platelet function disorders (IPFD) are a group of diseases with unclear mechanisms of hemorrhagic complications. There is limited and conflicting information on platelet properties in IPFD except for a limited number of classical mutations.
Aims: To evaluate CD62p (P-selectin) and phosphatidylserine (PS), markers of intravascular platelet activation associated with an impaired platelet function reactivity in IPFD.
Methods: 50 pediatric patients with diagnosed IPFD and hemorrhagic symptoms were examined. The median age was 7.5 years (range 0.9-18 years). The gender composition was 23 boys and 27 girls. All patients have bleeding of different severity: scoring from 2 to 16 according to Bleeding Assessment Tool ISTH. They have different hemorrhagic phenotype: petechiae and ecchymosis, nosebleeds, bleeding after tooth extraction, menorrhagia, hemarthrosis and subarachnoid hemorrhages. We investigated platelets in diluted blood using flow cytometry at rest and after activation with CRP plus TRAP-6. Control group (CG) included 50 healthy children (median age was 10 years (range 2-17 years). The analyses of statistical differences were performed using a nonparametric Mann-Whitney test.
Results: Forward scattering in patients wasn’t significantly different from the CG at rest and after activation (p = 0.43 and p = 0.24, respectively, Figure A, B). CD62p at rest was significantly higher in patients when compared with a group of healthy volunteers (p < 0.001), but no significant differences were observed after activation (p =0.21) (Figure C, D). PS at rest was significantly higher in patients (p < 0.001), but after activation was significantly lower in patients when compared with the CG (p = 0.035) (Figure E, F).
Conclusions: A significant increase of CD62p and PS at rest may indicate intravascular activation of platelets in patients, which may cause a decrease in overall functional reactivity upon activation contributing to the disease pathophysiology. This is in line with decrease of PS expression in patients after activation.
To cite this abstract in AMA style:Polokhov D, Ignatova A, Ponomarenko E, Zharkov P, Fedorova D, Novichkova G, Panteleev M. Platelet Activation Markers and Impaired Procoagulant Response in Pediatric Patients with Inherited Platelet Function Disorders [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/platelet-activation-markers-and-impaired-procoagulant-response-in-pediatric-patients-with-inherited-platelet-function-disorders/. Accessed October 1, 2023.
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