Advances in diagnosis and treatment of thrombotic thrombocytopenic purpura
Thrombotic thrombocytopenic purpura(TTP)is a life-threatening thrombotic disorder that has attracted widespread attention in recent years.The pathogenesis of TTP is associated with the deficiency of ADAMTS13,a von Willebrand factor-cleaving protease.The diagnosis of TTP primarily relies on clinical mani-festations and measurement of ADAMTS13 activity in plasma.The main differential diagnoses of TTP are hemo-lytic uremic syndrome,other types of thrombotic microangiopathy,and disseminated intravascular coagulation.Currently,plasma exchange and treatment with rituximab are the mainstays of TTP management.In addition,new therapeutic targets have emerged,such as caplacizumab,N-acetylcysteine,bortezomib,and recombinant ADAMTS13.Looking ahead,with further understanding of the mechanisms underlying TTP and ongoing clinical trials of new drugs,it is anticipated that more effective diagnostic and therapeutic approaches will be developed.Non-invasive oral medications may gradually replace invasive plasma exchange as a treatment option.
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