Development and validation of a predictive model for death in acquired severe ADAMTS13 deficiency-associated idiopathic thrombotic thrombocytopenic purpura: the French TMA Reference Center experience

Background. Acquired thrombotic thrombocytopenic purpura is still associated with a 10-20% death rate. So far, early prognostic factors of death could not be clearly identified. To identify prognostic factors associated with 1-month death in thrombotic thrombocytopenic purpura patients with acquired severe ( =10N. A 3-level risk score for early death was defined and confirmed in the validation cohort using these variables, with higher values corresponding to increased risk of early death.Conclusions. A risk score for early death was defined in patients with thrombotic thrombocytopenic purpura and validated on an independent cohort. This score should help to stratify early treatment and intensify patients with a worse prognosis.