The thrombotic-thrombocytopenic purpura (TTP) is an acute, life-threatening 
disease, characterised by enhanced platelet aggregation, disturbed 
microcirculation and organ dysfunction. With the currently available treatment 
(plasma exchange, infusions, corticosteroids) mortality ist still as high as 
10-15 %. Recent, pathophysiology-based developments may improve the outcome. The 
most promising candidates for future treatment of TTP are: rituximab for 
termination of the autoimmune process, caplacizumab for prevention of 
platelet-VWF-interaction, and recombinant ADAMTS13 for replacement of the 
inhibited or missing enzyme.