Aetiology and pathogenesis of thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome: the role of von Willebrand factor-cleaving protease

M Furlan, B Lämmle - Best practice & research Clinical haematology, 2001 - Elsevier
Thrombotic thrombocytopenic purpura (TTP) and haemolytic uraemic syndrome (HUS) are
today often regarded as variants of one syndrome denoted as TTP/HUS, characterized by
thrombocytopenia caused by intravascular platelet clumping, microangiopathic haemolytic
anaemia, fever, renal abnormalities and neurological disturbances. Unusually large von
Willebrand factor multimers have been observed in plasma from patients with chronic
relapsing forms of TTP. Their appearance in patients with classic TTP is caused by …