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Immune-mediated thrombotic thrombocytopenic purpura plasma induces calcium- and IgG-dependent endothelial activation: correlations with disease severity

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is characterized by a severe ADAMTS13 deficiency due to the presence of anti-ADAMTS13 auto-antibodies, with subsequent accumulation of circulating ultra-large von Willebrand factor (VWF) multimers. The role of endothelial cell activation as...

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Autors principals: Edwige Tellier, Agnès Widemann, Raphaël Cauchois, Julien Faccini, Marie Lagarde, Marion Brun, Philippe Robert, Stéphane Robert, Richard Bachelier, Pascale Poullin, Elien Roose, Karen Vanhoorelbeke, Paul Coppo, Françoise Dignat-George, Gilles Kaplanski
Format: Artigo
Idioma:Inglês
Publicat: Ferrata Storti Foundation 2022-12-01
Col·lecció:Haematologica
Accés en línia:https://haematologica.org/article/view/10884
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