Lymphocyte subpopulations in primary immunodeficiency disorders.
Venous blood mononuclear cells from 42 children with primary immunodeficiency disorders and from controls matched for age were studied for lymphocyte subpopulations by E rosetting, surface immunoglobulin, and a panel of anti T cell monoclonal antibodies (OKT series). In 3 cases of severe combined immunodeficiency (SCID) due to adenosine deaminase deficiency, very few circulating T or B cells were found. The other 7 cases of SCID all had normal or, in 3 cases, very high numbers of circulating B cells, but in 6 of these very few cells showed T cell markers. One child had very high numbers of B cells and T cells with an immature pattern of reactivity similar to that found on common thymocytes. In T cell deficient children no consistent pattern was found, but in those with cartilage hair hypoplasia with immunodeficiency there was a low helper (OKT4) to suppressor (OKT8) ratio and high numbers of circulating OKT10 positive cells. In cases of X-linked agammaglobulinaemia circulating B cells were not found but the pattern of T cell markers was normal. In cases of common variable hypogammaglobulinaemia there was a wide scatter of helper (OKT4) to suppressor (OKT8) cell ratios. Five children were studied before and after treatment with the synthetic thymic hormone preparation TP5. There were appreciable alterations in the pattern of staining with anti T cell monoclonal antibodies in 4 of these cases, but in 1 case only was this accompanied by improvements in clinical and immune function.
D000361:Agammaglobulinemia / Q000276:immunology; D001402:B-Lymphocytes / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000188:drug therapy / Q000276:immunology*; D007223:Infant; D007231:Infant, Newborn; D007958:Leukocyte Count; D008214:Lymphocytes / Q000145:classification*; D008297:Male; D010446:Peptide Fragments / Q000627:therapeutic use; D012397:Rosette Formation; D013601:T-Lymphocytes / Q000145:classification / Q000187:drug effects / Q000276:immunology; D016305:Thymopentin; D013946:Thymopoietins / Q000627:therapeutic use
D010446:Peptide Fragments; D013946:Thymopoietins; D016305:Thymopentin