Complement and Complement Component Activities in Diseases Associated with Repeated Infections and Malignancy

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Abstract
Methods were adapted for assay of total C and C component hemolytic activities, immune adherence C activity and bactericidal C activity in whole human serum; and normal values for newborns, children and adults were established. The C titers were decreased in the newly born (C''1 disproportionately so) with adolescent levels reached by 2. No differences were seen according to sex in any age group. Patients with both nonlymphopenic and lymphopenic forms of agammaglobulinemia had significantly decreased titers of C''l, but normal or elevated titers of total C, C''4, C''2 and C''3t hemolytic activities. Immune adherence C and bactericidal C titers also were normal. Neutrophil chemotaxis was normal in nonlymphopenic agammaglobulinemia, but questionable in lymphopenic agammaglobulinemia. Chickens with experimental agammaglobulinemia and bovine embryos as early as the 12 cm stage with developmental agammaglobulinemia both exhibited development of C function. Normal C and C component titers were observed in patients with a variety of hereditary diseases associated with repeated infections and malignancy Chediak-Higashi syndrome, Aldrich syndrome, cystic fibrosis of the pancreas, Riley-Day syndrome, ataxia-telangiectasia, and fatal granulomatous disease of childhood. The C function and C components C''2-C''9 can develop and persist independent of the 2 defined lymphoid systems of birds and mammals. However, a deficiency of C''l, and in some lymphopenic forms at least its C''1q subcomponent, is linked with the deficiency of immunoglobulin synthesis in the several agammaglobulinemia syndromes.