Four siblings with recurrent bacterial infections, neutrophil chemotactic defect, neutropenia, and eosinophilia were studied. During periods of infection the peripheral neutrophil count increased to normal, while the eosinophilia disappeared. In addition, these children had high levels of serum IgA and poor antibody responses to tetanus and polio vaccinations. A defect in cell-mediated immunity was demonstrated by an absent or weak reactivity to various skin test antigens and by abnormal lymph node histology. Thus these siblings had an unusual combination of defective inflammatory response and immunologic abnormalities.

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Summary An instance of familial leukaemia is described where 3 children out of 4 died of acute lymphocytic leukaemia at the ages of 2–4 years. Two of the siblings were twins, probably binovular. Genetic mechanisms in the aetiology of acute leukaemia are discussed and the pertinent literature is briefly reviewed.

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