Acquired thrombotic thrombocytopenic purpura (aTTP), is a rare disease caused by ADAMTS13 deficiency, with an annual incidence of 1-6 million/inhabitants. Despite the effectiveness of treatment with therapeutic plasma exchange (TPE) and immunosuppressants, aTTP is still associated with a 10-20% death rate, and its clinical course is characterized by recurrent episodes in up to 50% of cases. Early recognition of patients at higher risk of mortality has become of paramount importance since new treatments such as caplacizumab could ameliorate the prognosis of this group. Over the last decade, mortality predicting models like the French TMA Reference Center Score (FTRCC) and the Mortality in TTP Score (MITS) have been developed in an attempt to personalize treatment. In a recently published study, they were applied to clinical practice to characterize first and relapsed aTTP episodes. This commentary discusses the historical evolution of aTTP prognostic scores and their current role in predicting the prognosis of first episodes and relapses of aTTP.
Thrombotic thrombocytopenic purpura, Mortality, Scores prognostic, ADAMTS13