Nephrol Dial Transplant (2000) 15: 1112-1114
© 2000 European Renal Association-European Dialysis and Transplant Association
Editorial Comments
Haemolytic-uraemic syndrome and thrombotic thrombocytopenic purpura—new insights into underlying biochemical mechanisms
Central Haematology Laboratory, University Hospital, Inselspital, Bern, Switzerland
Introduction
Haemolytic-uraemic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are two clinically similar disorders characterized by severe microangiopathic haemolytic anaemia and thrombocytopenia. HUS is characterized by thrombocytopenia, anaemia and renal insufficiency, whereas the pentad of signs and symptoms including thrombocytopenia, anaemia, neurologic deficit, renal dysfunction and fever is observed in TTP. However, about 60% of patients diagnosed with acute TTP lack one or more of these criteria, while about 30% of those receiving a diagnosis of HUS exhibit neurologic symptoms and fever. Thus, the two disorders are often difficult to distinguish. Given the lack of consistent clinical findings, the same disorder might be diagnosed as HUS by a nephrologist while a haematologist might call it TTP. Since both HUS and TTP have been reported to occur in siblings and on different occasions even in the same patient, it sometimes appears that the two disorders are different manifestations of the same pathophysiologic
Endothelial cell activation in HUS
Proteolytic cleavage of von Willebrand factor
Deficiency of von Willebrand factor-cleaving protease in TTP
Normal activity of von Willebrand factor-cleaving protease in HUS
Management of HUS and TTP
Acknowledgments
Notes
References