Diagnostic challenges of Thrombotic Thrombocytopenic Purpura: Results from a cohort of 3,100 individual patients in a time period of 21 months

Introduction Thrombotic Thrombocytopenic Purpura (TTP) is a life threatening microangiopathy, due to severe deficiency of the von Willebrand factor (VWF) cleaving protease ADAMTS13, which can either be caused by a genetic defect of the ADAMTS13 gene or much more often, by autoantibodies against the protease. TTP is diagnosed by characteristic clinical symptoms of microangiopathy, the presence of schistocytes in the blood smear, thrombocytopenia, elevated LDH and finally, deficiency of ADAMTS13.