Article
Child-onset and adolescent-onset acquired thrombotic thrombocytopenic purpura with severe ADAMTS13 deficiency: a cohort study of the French national registry for thrombotic microangiopathy.
The Lancet. Haematology - 1 Nov 2016
Joly Bérangère S, Stepanian Alain, Leblanc Thierry, Hajage David, Chambost Hervé, Harambat Jérôme, Fouyssac Fanny, Guigonis Vincent, Leverger Guy, Ulinski Tim, Kwon Thérésa, Loirat Chantal, Coppo Paul, Veyradier Agnès
Abstract excerpt
BACKGROUND: Thrombotic thrombocytopenic purpura is a rare thrombotic microangiopathy, related to a severe ADAMTS13 deficiency (a disintegrin and metalloprotease with thromboSpondin type 1 repeats, member 13; activity <10% of normal). Childhood-onset thrombotic thrombocytopenic purpura is very rare and initially often misdiagnosed, especially when ADAMTS13 deficiency is acquired (ie, not linked to inherited...
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