While haemolytic uraemic syndrome in children is predominantly associated with Shiga toxin -producing Escherichia coli (typically 0157:H7), some cases occur without associated diarrhoea, or as the manifestation of an underlying disorder other than infection.
Haemolytic uraemic syndrome is characterised
by microangiopathic anaemia, thrombocytopaenia
and renal failure, on occasion accompanied by severe hypertension. Malignant hypertension is a syndrome that sometimes exhibits the same laboratory abnormalities
as haemolytic uraemic syndrome as it may
share the same pathological findings: thrombotic microangiopathy. As clinical features of both entities overlap, the distinction between them can be very
difficult. However, differentiation is essential for the treatment decision, since early plasma exchange dramatically reduces mortality in haemolytic uraemic syndrome not associated with diarrhoea. An increasing
number of genetic causes of this pathology have been described and may be very useful in differentiating it from thrombotic microangiopathy due to other aetiologies. Despite advances in the understanding
of the pathophysiology of haemolytic uraemic
syndrome not associated with diarrhoea, the
management often remains empirical. We describe a patient with simultaneous microangiopathic haemolytic
anaemia, thrombocytopaenia and severe hypertension managed in the acute period of illness with plasma exchange
