• S Audia, M Mahevas, and B Bonnotte.
• Service de Médecine Interne et Immunologie Clinique, Médecine 1-SOC 1, Hôpital François Mitterrand, Centre de référence des cytopénies auto-immunes de l'adulte, CHU Dijon-Bourgogne, 14 rue Paul Gaffarel, 21079 Dijon, France; Unité RIGHT, INSERM UMR 1098, Équipe "Immunorégulation et immunopathologie", Bâtiment B3, 15 rue Maréchal de Lattre de Tassigny, 21000 Dijon, France. Electronic address: Sylvain.audia@u-bourgogne.fr.
• Rev Med Interne. 2021 Jan 1; 42 (1): 16-24.

AbstractImmune thrombocytopenia (ITP) is a rare autoimmune disease due to an immune peripheral destruction of platelets and an inappropriate platelet production. The pathogenesis of ITP is now better understood: it involves a humoral immune response which dependents on the stimulation of B cells by specific T cells called T follicular helper cells, leading to their differentiation into plasma cells that produce antiplatelet antibodies thus promoting the phagocytosis of platelets mainly by splenic macrophages. The deciphering of ITP pathogenesis has led to a better understanding of the inefficiency of treatments such as rituximab, although it has not provided yet the determination of biological predictive factor of response to treatments. Moreover, new therapeutic perspectives have been opened in the last few years with the development of molecules targeting Fcγ receptor signalling such as Syk inhibitor, or molecules increasing the clearance of pathogenic autoantibodies such as inhibitors of the neonatal Fc receptor (FcRn).Copyright © 2020. Published by Elsevier Masson SAS.

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