Sex-linked recessive disease of infants characterized by haemorrhagic diathesis, eczema and recurrent infections. Delayed hypersensitivity reactions are absent and there is a defective antibody response to polysaccharide antigens, with low blood IgM levels. A protein normally present in platelet membranes is missing. This combined defect of cell- mediated and humoral immunity probably involves failure to recognize or process antigens. Patients die early of infection or bleeding due to increased destruction of platelets.
What is WISKOTT-ALDRICH SYNDROME?
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