Thrombotic thrombocytopenic purpura (TTP) and haemolytic uraemic syndrome (HUS) are thrombotic microangiopathies that present acutely and have been associated with high morbidity and mortality. Presented is the current understanding of the pathophysiology of these disorders, but also the benefit of newer treatments in improving the acute and long-term outcomes of these rare conditions. In immune TTP, the addition of caplacizumab to the treatment pathways and the prospect of recombinant ADAMTS 13 in the future. In congenital TTP, the benefit of recombinant ADAMTS13 over standard plasma-based treatments in reducing symptoms and burden of therapy. Finally, in HUS, the role of the C5 complement inhibitors, eculizumab and ravulizumab and the plethora of new complement inhibitors under investigation for the future.