Wiskott-Aldrich syndrome: life-threatening haemorrhage from aneurysms within the liver, small bowel mesentery and kidney, requiring both surgical and radiological intervention.
2000
Wiskott-Aldrich syndrome (WAS) is a rare, generally X-linked recessive condition, originally described by Wiskott in 1937 1 as a triad of discharging ears, eczema and thrombocytopoenia. Aldrich included bloody diarrhoea in his report of 1954 2 , with severe immunodeficiency and predisposition to malignancy being recognised subsequently. The incidence currently quoted is approximately 4 per million live male births, although there is some regional variation 3 . We report the case of a long-term survivor who had massive haemorrhage from an intra-hepatic aneurysm and, on a separate occasion, the right kidney.
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