Wiskott-Aldrich syndrome: life-threatening haemorrhage from aneurysms within the liver, small bowel mesentery and kidney, requiring both surgical andradiological intervention

Wiskott-Aldrich syndrome (WAS) is a rare, generally X-Iinked recessive cond ition, originally described by Wiskott in 1937(1) as a triad of discharging ears, eczema and thrombocytopoenia. Aldrich included bloody diarrhoea in h is report of 1954(2), with severe immunodeficiency and predisposition to ma lignancy being recognised subsequently. The incidence currently quoted is a pproximately 4 per million live male births, although there is some regiona l variation(3). We report the case of a long-term survivor who had massive haemorrhage from an intrahepatic aneurysm and, on a separate occasion, the right kidney.