2 results match your criteria: "Centre de référence constitutif des microangiopathies thrombotiques[Affiliation]"

[Treatment of immune-mediated thrombotic thrombocytopenic purpura: A decisive turning point].

Transfus Clin Biol

November 2021

Service d'hémaphérèse, Centre de référence constitutif des microangiopathies thrombotiques, hôpital de la Conception, 147, boulevard Baille, 13385 Marseille cedex 5, France. Electronic address:

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare and life-threatening thrombotic microangiopathy characterized by severe deficiency of ADAMTS13, the enzyme that cleaves von Willebrand factor multimers. Recent insights into iTTP pathophysiology have led to the development of new therapies targeting ADAMTS13 replacement, anti-ADAMTS13 antibodies, and von Willebrand factor-platelet interactions. New maximalist therapeutic strategies are emerging based on triple therapy.

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Background: Octaplas LG® is the first plasma with marketing authorisation, available in France only since February 2016. This is a double viral inactivated and prion reduced solvent/detergent fresh frozen plasma. Clinical data on Octaplas LG® use in thrombotic microangiopathy (TMA) remains very limited.

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