Daily therapeutic plasma exchange (TPE) transformed the historically fatal 
prognosis of acquired, anti-ADAMTS13 antibody-mediated thrombotic 
thrombocytopenic purpura (TTP), leading to the current overall survival rates of 
>80%. However, relapses occur in up to 40% of patients and refractory disease 
with fatal outcomes still occurs, typically within the first days of management. 
In this context, the introduction of rituximab has been the second major 
breakthrough in TTP management. Rituximab is now routinely recommended during 
the acute phase, typically in patients with a suboptimal response to treatment, 
and increasingly as frontline therapy, with high response rates in the following 
weeks. In more severe patients, salvage strategies typically include twice daily 
TPE, pulses of cyclophosphamide, as well as splenectomy in the more desperate 
cases. In this life-threatening and debilitating disease, relapses can be 
efficiently prevented in patients with a severe acquired ADAMTS13 deficiency and 
otherwise in remission with the use of rituximab. In the coming years, the TTP 
therapeutic landscape should be enriched by original strategies stemming from 
clinical experience and new agents that are currently being evaluated in large, 
international, clinical trials. Promising agents under evaluation include 
caplacizumab (an inhibitor of the glycoprotein-Ib/IX-Von-Willebrand factor 
axis), N-acetylcysteine, recombinant ADAMTS13, and anti-plasmocyte compounds.