Abstract
Lipoprotein complexes have been associated with depressed immunologic functions. An 18-month old male presented with a history of intermittent and recurrent fevers, hepatosplenomegaly, anemia and thrombocytopenia. The patient developed a progressive polymyositis associated with elevations in CPK and aldolase enzymes; muscle biopsy revealed infiltration and replacement of striated muscles with connective tissue and mononuclear cells. Analysis of serum demonstrated a hyperlipidemia consistent with a Type I phenotype. Although the patient demonstrated only minimal depression of serum Ig levels, the addition of his serum to cultures of healthy donor peripheral blood lymphocytes (PBL) stimulated with pokeweed mitogen resulted in significant suppression of Ig synthesis and secretion. This suppressor activity was also demonstrated when washed patient PBL were cocultured with normal donor cells in the absence of patient serum. Furthermore, suppressor activity correlated with increased levels of T cells bearing surface receptors for the Fc portion of IgG (Tγ cells). These results support a model which includes the role of lipoproteins in the modulation of the immune response.
(Supported in part by NIH grants CA-08748, CA-17404 and CA-19267 and American Cancer Society grant IM-126.)
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Schwartz, S., Gupta, S. & Good, R. 736 ASSOCIATION OF MYOSITIS, HYPERLIPIDEMIA AND IMMUNO-REGULATORY DYSFUNCTION. Pediatr Res 12 (Suppl 4), 486 (1978). https://doi.org/10.1203/00006450-197804001-00741
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DOI: https://doi.org/10.1203/00006450-197804001-00741