The role of the endothelium in thrombotic thrombocytopaenic purpura and haemolytic uraemic syndrome

Thrombotic thrombocytopaenic purpura (TTP) is an uncommon and severe multi-system disease characterized by intravascular platelet aggregation, thrombocytopaenia, central nervous system (and other organ) ischaemia and microangiopathic haemolytic anaemia that mainly occurs in adults (Bukowski, 1982). TTP shows many similarities to haemolytic uraemic syndrome (HUS), a disorder of thrombocytopaenia, microangiopathic haemolytic anaemia and acute renal failure that mainly affects young children (Kaplan & Remuzzi, 1991).