Article
Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura): a cross-sectional analysis of the French national registry for thrombotic microangiopathy.
The Lancet. Haematology - 1 May 2016
Mariotte Eric, Azoulay Elie, Galicier Lionel, Rondeau Eric, Zouiti Fouzia, Boisseau Pierre, Poullin Pascale, de Maistre Emmanuel, Provôt François, Delmas Yahsou, Perez Pierre, Benhamou Ygal, Stepanian Alain, Coppo Paul, Veyradier Agnès
Abstract excerpt
BACKGROUND: Thrombotic thrombocytopenic purpura is a thrombotic microangiopathy related to a severe deficiency of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13; activity <10%). We aimed to investigate the association between mechanisms for ADAMTS13 deficiency and the epidemiology and pathophysiology of thrombotic thrombocytopenic purpura at initial presentation....
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