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A Subpopulation of ITP Patients Resembling Glanzmann Thrombasthenia Phenotype Revealed by Flow Cytometry Analysis of Platelet Intracellular Signalling

A. Martyanov1,2,3, D.S. Morozova4, S.S. Uzueva3, M.A. Sorokina3, D.V. Fedorova3, P.A. Zharkov3, M.A. Panteleev2,3,5, A.N. Sveshnikova2,3,5

1Institute for Biochemical Physics, Moscow, Russian Federation, 2Centre for Theoretical Problems of Physicochemical Pharmacology, Moscow, Russian Federation, 3National Medical Research Centre of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow, Russian Federation, 4Lomonosov Moscow State University, Faculty of Medicine, Moscow, Russian Federation, 5Lomonosov Moscow State University, Faculty of Physics, Moscow, Russian Federation

Abstract Number: PB1369

Meeting: ISTH 2020 Congress

Theme: Platelet Disorders and von Willebrand Disease » Acquired Thrombocytopenias

Background: Immune thrombocytopenia (ITP) is an autoimmune condition characterized by isolated low platelet count, primarily induced by the loss of immune tolerance to the platelet glycoproteins. Different auto-antibodies may affect platelet function in addition to the platelet count. Distinguishing between the different ITP causes would facilitate the appropriate therapy selection.

Aims: Comparison of platelet calcium signalling, integrin activation and thrombus formation between ITP patients, Glanzmann thrombasthenia patients and healthy donors.

Methods: Whole hirudinated blood of healthy pediatric donors (CNT; n = 11) or pediatric patients with immune thrombocytopenia (ITP; n=36) and Glanzmann thrombastenia (GT; n=8) within 3 hours of collection was used in the experiments. Continues flow cytometry was used for characterization of calcium signalling and fibrinogen binding in quiescent platelets or in response to ADP, collagen-related peptide or TRAP. All numbers are given for ADP stimulation. Calcium levels are given in nM, fibrinogen binding is given in percentage of total integrin activation. Parallel-plate flow chambers were used for observation of thrombus formation. Numerical and statistical analysis was performed in Python 3.7.

Results: All ITP patients had increased basal platelet calcium concentrations (15±5(ITP) vs 8±5(CNT)). Clustering analysis of the flow cytometry revealed two subpopulations of ITP patients: the high fibrinogen binding (HFB) group with comparable to CNT fibrinogen binding and higher calcium mobilization (154±27 (HFB) vs 92±22(CNT)), and the low fibrinogen binding (LFB) group with lower fibrinogen binding (8±5 (LFB) vs 16±3(CNT)). GT platelets had calcium mobilization (81±23), fibrinogen-binding (5.1±0.3) and thrombus growth comparable to LFB. Two computational models of platelet intracellular signalling leading to integrin activation for HFB and LFB were developed.

Conclusions: The group of ITP patients with GT-like phenotype was revealed. This group is characterized by low integrin activation, high quiescent platelet calcium and thrombus growth impairment. The systems-biology analysis suggested weakened integrin activation for this population.

To cite this abstract in AMA style:

Martyanov A, Morozova DS, Uzueva SS, Sorokina MA, Fedorova DV, Zharkov PA, Panteleev MA, Sveshnikova AN. A Subpopulation of ITP Patients Resembling Glanzmann Thrombasthenia Phenotype Revealed by Flow Cytometry Analysis of Platelet Intracellular Signalling [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/a-subpopulation-of-itp-patients-resembling-glanzmann-thrombasthenia-phenotype-revealed-by-flow-cytometry-analysis-of-platelet-intracellular-signalling/. Accessed October 1, 2023.

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