Abstract
Associations of specific histocompatibility antigens with certain human diseases may elucidate basic mechanisms of disease. Since there is evidence for a disordered immune response in MLNS, HLA antigens were evaluated by NIGH standard technique in 27 patients (3 Oriental, 1 black) fulfilling CDC criteria for MLNS. In contrast to previous Japanese studies, we found no incidence of HLA-Bw22. We did, however, find a significant increase in HLA-Bw51: CHANCES OF DISTRIBUTION OF HLA PHENOTYPES IN CAUCASIANS
To our knowledge, this is only the second disease associated with HLA-B5 specificity. In preliminary studies, 10/17 Caucasian patients with MLNS were DR-2 (frequency in control population 23%, p<0.01). In other studies (Geha, et al), suppressor T-cells(OKT8) were found decreased in the acute phase of MLNS. Thus, MLNS, like systemic lupus erythmatosus and multiple sclerosis, is associated with both decreased suppressor cells and DR-2. Such findings may have genetic implications regarding inter-human variation in immune responsiveness. Infectious agents, toxins, or endogenous immune stimulation may trigger, in genetically susceptible persons, an inappropriate immune response.
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Krensky, A., Berenberg, W., Grady, S. et al. 735 HLA ANTIGENS IN MUCOCUTANEOUS LYMPH NODE SYNDROME (MLNS). Pediatr Res 15 (Suppl 4), 564 (1981). https://doi.org/10.1203/00006450-198104001-00758
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DOI: https://doi.org/10.1203/00006450-198104001-00758