Biochemical Abnormalities in the Wiskott-Aldrich Syndrome: Low Activity of Hexokinase in Lymphocytes

Two patients with Wiskott-Aldrich syndrome were studied immunologically and biochemically. The same immunological abnormalities were found in these patients as described in the previous cases. Futher, the expression of IgG-Fc receptor on the surface of monocytes was poor and anti-D coated human erythrocytes were less phagocytized by these cells from the patients than by control cells. The hexokinase activity was low in mononuclear cells (30% of the control mean) but normal in erythrocytes. It was suggested that a disturbance of the energy metabolism may affect the antigen processing mechanism and the afferent limb of immunity in the immunocompetent cells in this disease.