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Thrombotic thrombocytopenic purpura related to severe ADAMTS13 deficiency in children

✍ Scribed by Chantal Loirat; Jean-Pierre Girma; Céline Desconclois; Paul Coppo; Agnès Veyradier

Publisher
Springer
Year
2009
Tongue
English
Weight
277 KB
Volume
24
Category
Article
ISSN
0931-041X

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## Abstract Arterial thrombotic events, thrombocytopenia, and hemolytic anemia with schistocytes may be encountered in the setting of both thrombotic thrombocytopenic purpura (TTP) and primary antiphospholipid syndrome (APS). We report 2 cases of TTP occurring in patients with definite primary APS.

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Congenital thrombotic thrombocytopenic purpura (TTP) (also known as Upshaw-Schulman syndrome, USS) is a rare, life-threatening disease characterized by thrombocytopenia and microangiopathic hemolytic anemia, associated with the deficiency of the von Willebrand factor-cleaving protease (ADAMTS13) due