Wiskott-Aldrich syndrome: a multidisciplinary disease.

Introduction Wiskott-Aldrich syndrome is a rare X-linked disorder which, in its fully expressed form, is recognised by the clinical triad of combined immune deficiency, thrombocytopenia, and eczema.' An increased risk of malignancy has also been reported, with an incidence of about 12% in some series and susceptibility, in particular, to acute leukaemia, lymphoma, and solid tumours of the central nervous system.2 Other less common manifestations include asthma, autoimmune haemolytic anaemia, arthritis and glomerulonephritis. Because of the combined risks of serious infective complications, bleeding and malignancy, survival to adulthood is rare. There is some optimism for the future prospects of patients with the syndrome and their families, however, as a result of recent advances in the molecular biology and genetics of the disorder and parallel developments in clinical management.