Flowchart for the therapeutic management of adult-onset acquired TTP. The standard treatment of adult-onset acute acquired TTP is daily therapeutic plasma exchange (±steroids) initiated in emergency. This first-line treatment may induce a complete response, but some patients may also be unresponsive, or they may exhibit an exacerbation. In both these latter cases, twice-daily plasma exchange may be prescribed together with rituximab, with this second-line treatment usually leading to complete remission. Follow-up of patients in remission (consisting of medical consultation with standard biology and ADAMTS13 monitoring) may show either a durable remission or relapses requiring standard treatment (with variable outcomes similar to those of the inaugural acute event). In some cases, ADAMTS13 monitoring allows us to identify a decrease of ADAMTS13 activity less than 10%, in the absence of clinical relapse. Considering the high risk for relapse in this situation, it is reasonable to consider a preemptive treatment with rituximab.