Abstract
AbstractThrombotic thrombocytopenic purpura is characterized by a severe deficiency in ADAMTS13 (A disintegrin and metalloprotease with a thromboSpondin type 1 repeats, member 13) activity, lower than 10%. This disease involves Von Willebrand Factor (VWF), which is not cleaved by ADAMTS13. Deficiency is mainly acquired, due to anti-ADAMTS1 3 antibodies (iPTT). Mechanical haemolytic anemia, thrombocytopenia due to consumption and thrombosis in microvessels are observed. The diagnosis should be quickly discussed and confirmed by the measurement of ADAMTS13 activity. Pending the results of this marker, clinicians can use scores with high positive predictive values, which help them rapidly introduce replacement therapies, given the poor prognosis of TTP in the absence of treatment. Caplacizumab is also used as a first-line treatment to limit the formation of microthrombi, pending the efficacy of immunosuppressive treatments started at the same time. Specialized management and long-term follow-up of patients is crucial to evaluate sequelae and to predict relapses.
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