Abstract:Thrombotic thombocytopenic purpura (TTP) is characterized by a reduction in the von Willebrand cleavage protein ADAMTS-13, caused mainly by autoimmunity. Plasmapheresis is the standard of care achieving complete remission (CR) in 77-83% of cases but figures are variable depending on clinical context and ADAMTS-13 activity. Relapse is frequent in patients with <10% ADAMTS-13, and has been reported in 34-41% of cases. In most of these patients, an inhibitor against ADAMS-13 is detected thus, an effective fron… Show more
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