Haemolytic uraemic syndrome and thrombotic thrombocytopenic purpura

Abstract 

This review will focus on the classification, symptoms, pathology, pathogenesis and management of haemolytic uraemic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). HUS is characterized by microangiopathic haemolytic anaemia, thrombocytopenia and acute renal failure. Typical cases have been associated with infections caused by Shiga toxin (Stx)-producing bacteria manifested by a diarrhoeal prodrome. Atypical cases have heterogeneous aetiologies not associated with Stx. Certain cases may be hereditary and mutations in complement factor H have been identified in a subset of these patients. TTP is a similar condition characterized by microangiopathic haemolytic anaemia, thrombocytopenia, nephropathy, fever and neurological symptoms associated with mutations in the von Willebrand cleaving protease. The pathological lesion has been termed thrombotic microangiopathy revealing small vessel lesions and platelet thrombosis leading to obstruction of blood flow. Supportive management reduces morbidity and mortality. Patients with atypical HUS and TTP may benefit from specific treatment with plasma infusions or exchange.

Keywords: haemolytic uraemic syndrome, thrombotic thrombocytopenic purpura, Shiga toxin, factor H, von Willebrand factor, paediatric

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• * Correspondence to: DK. Tel.: +46-46-2220745, Fax: +46-46-2220748; E-mail: diana.karpman@pedi.lu.se