[Antigammaglobulin factors and malignant diseases].
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Biomedical subjects
Publications and source records attributed to F Tedesco.
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A 15-year-old woman with a history of recurrent episodes of meningococcal infections was admitted to our hospital with signs and symptoms indicating a meningeal inflammation. Since in the last few years some of the patients affected by recurrent meningococcal infections have been recognized to have selective complement deficiencies, the patient's serum was studied for determining the complement function. C8 was found to be present only in traces, with a pattern of partial identity as compared with that of the normal human serum. Moreover, total hemolytic complement was undetectable and could be completely restored with purified C8, but not with other complement components; thus, we concluded for the presence of a C8 deficiency state. Further reconstitution experiments carried out with sera having selective deficiencies of either C8 beta or C8 alpha-gamma subunits allowed us to recognize the presence of a dysfunctional C8 molecule lacking the beta chain, but possessing the alpha-gamma subunit. The clinical history of the patient, characterized by recurrent meningococcal infections, further supports the current concept of an increased susceptibility of the C8 beta-deficient patients to Neisseria meningitidis infections.
Seven pediatric patients with monoarticular arthritis, three of whom had a recent onset form and the remaining four a disease of longer duration, were examined for possible modifications of their immunological parameters. The diagnosis of JRA was made on all these patients according to the ARA criteria after a follow-up of at least two years. Humoral and cellular abnormalities of the immune system were searched for in peripheral blood, synovial fluid and synovial membrane. No evidence for complement consumption and for increased levels of immune-complexes was found in the sera and in the synovial fluids of these patients, who were all seronegative. Some patients had antinuclear antibodies in their sera and synovial fluids. With regard to the lymphocyte distribution, whereas only some patients had an increased number of circulating B cells, the majority had a decreased CD4+/CD8+ ratio in the synovial fluid compared to the ratio found in the peripheral blood. A massive infiltration of CD4+ cells and macrophages and the presence of a substantial number of OKT9+ cells was found in the synovial membranes.
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