If TTP is confirmed by ADAMTS-13 activity ≤ 10%, presence of ADAMTS-13 autoantibodies and no family history suggestive of an Upshaw-Schulman syndrome, corticosteroid therapy with the addition of rituximab is recommended during both first and relapse episodes [13]. Here, ADAMTS13 is linked to congenital thrombotic thrombocytopenic purpura.