Haemolytic uraemic syndrome / thrombocytopaenic thrombotic purpura
Keywords:
Endothelial dysfunction, factor H, haemolytic uraemic syndrome, plasmapheresis, thrombocytopenic thrombotic purpura, von Willebrand factorAbstract
Haemolytic uraemic syndrome and thrombocytopenic thrombotic
purpura are characterized by the presence of haemolytic microangiopathic anaemia and thrombocytopenia. The endothelial
dysfunction and the release of von Willebrand factor high molecular weight multimers are implicated in the sequence of events
that lead to microvascular thrombosis. The high plasma level of
lactic dehydrogenase is the most sensitive index for the ongoing microangiopathic haemolysis.
In haemolytic uraemic syndrome the microthrombi are confined
to the kidney, being the acute renal failure the dominant clinical
feature. Thrombocytopenic thrombotic purpura affects mainly
the brain, with intermittent thrombi formation and presenting
fl uctuating neurological symptoms.
The authors review the major characteristics, clinical features,
prognosis and treatment of the primary and secondary haemolytic
uraemic syndrome / thrombocytopenic thrombotic purpura forms,
as well as the acute, chronic, recurrent and plasmapheresis
resistant presentations.
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