title: Usefulness of monoclonal antibodies in the diagnosis and monitoring of patients with primary immunodeficiencies: combined experience in three clinical immunology centers.
abstract: Circulating levels of T-cell subsets and NK cells were determined in 78 patients with primary immunodeficiencies, 35 children with recurrent respiratory infections, and healthy age-matched controls. Normal T cell and natural killer (NK) cell values were observed in individuals with immunoglobulin A (IgA) deficiency and X-linked agammaglobulinemia, while reduced OKT4/OKT8 cell ratios and low levels of 5/9+ T helper cells were found in approximately 60% of patients with common variable immunodeficiency. Infants with severe combined immunodeficiency (SCID) and lymphopenia had virtually no cells expressing T-cell or NK-cell surface antigens, but had normal numbers of monocytes and other types of blood cells. Infants with DiGeorge syndrome, other primary T-cell defects, or SCID with B cells had few or no circulating cells of mature T helper-suppressor phenotypes, but had normal numbers of NK cells (HNK-1+) and NK function. These results support the idea of a common stem cell precursor for T, B, and NK cells, each of which follows a separate pathway of differentiation. Profound alterations were observed in the distribution and function of T-cell subsets in ataxia-telangiectasia patients who were previously shown to have thymic dysplasia. A significant reduction in the frequencies of OKT3+ and OKT4+ cells was observed in children with frequent respiratory infections during infancy.(ABSTRACT TRUNCATED AT 250 WORDS)
mesh_terms: D000361:Agammaglobulinemia / Q000276:immunology; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D001260:Ataxia Telangiectasia / Q000276:immunology; D001402:B-Lymphocytes / Q000276:immunology; D016026:Bone Marrow Transplantation; D002908:Chronic Disease; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000276:immunology* / Q000628:therapy; D007223:Infant; D007244:Infectious Mononucleosis / Q000276:immunology; D008297:Male; D010446:Peptide Fragments / Q000627:therapeutic use; D012008:Recurrence; D012141:Respiratory Tract Infections / Q000276:immunology; D013601:T-Lymphocytes / Q000276:immunology; D016305:Thymopentin; D013946:Thymopoietins / Q000627:therapeutic use; D014960:X Chromosome
chemical_list: D000911:Antibodies, Monoclonal; D010446:Peptide Fragments; D013946:Thymopoietins; D016305:Thymopentin