Active and suppressor T cells: diminution in a patient with dyskeratosis congenita and in first-degree relatives.
Active, total and nonspecific suppressor T cells were studied in a 15-year-old black male with dyskeratosis congenita syndrome, a precancerous mucosal disease, and in 7 siblings and several other relatives in three generations. The propositus and 1 elder sister, products of a second-cousin marriage, died with dyskeratosis congenita. The mother had dermatomyositis, and the maternal grandmother and her sister reportedly had rheumatoid arthritis. Studies of available siblings, father, and grandparents revealed a high incidence of deficiency in number of active and/or suppressor T cells, sometimes severe enough to result in a decrease in total T cells. The patient had many stigmata of precocious aging, as did the sibling who died with the same syndrome. The laboratory data suggest that a defect in cell-mediated immunity, involving mainly or exclusively suppressor T cells, is associated with, and is presumably the cause of, precocious aging; perhaps an abiotrophy in this cell subpopulation results in physiologic aging.
D000293:Adolescent; D000328:Adult; D000375:Aging; D005260:Female; D006801:Humans; D008231:Lymphopenia / Q000209:etiology; D008297:Male; D010375:Pedigree; D011247:Pregnancy; D012397:Rosette Formation; D012871:Skin Diseases / Q000151:congenital* / Q000235:genetics / Q000276:immunology; D013601:T-Lymphocytes / Q000502:physiology*