Thrombotic thrombocytopenic purpura (TTP) is characterized by microangiopathic 
hemolytic anemia and a consumptive thrombocytopenia, as a result of severe 
deficiency of ADAMTS13. The standard of care of the acute episode is treatment 
with plasma exchange and immunosuppression. After the acute episode is resolved, 
patients face a significant risk of relapse and long-term complications 
associated with significant morbidity and even mortality. Novel treatments have 
been under development and will be discussed in this review. Caplacizumab, a 
nanobody that blocks the interaction between VWF and platelets, has shown 
promising results in decreasing the time to recover from the acute events that 
will hopefully translate into long-term clinical benefit for patients. In 
addition, identifying biomarkers to allow us to better predict the risk for 
relapse and the development of these long-term complications in patients with 
TTP are a few of the challenges that require our attention moving forward.