title: B-cell and T-regulatory cell dysfunction in six Chinese children with hypogammaglobulinaemia.
abstract: We report six Chinese boys with hypogammaglobulinaemia. All but one had very low or undetectable circulating B-lymphocytes, two had reversed CD4/CD8 ratios (in one of whom this latter became normal), one had reduced lymphocyte proliferative responses to concanavalin A and pokeweed mitogen and three had deficient responses to OKT3. Generation of antibody-secreting cells in response to pokeweed mitogen was markedly defective in all patients. Co-cultures of purified lymphocyte subsets from the patients with those of normal donors revealed that in addition to B-cell deficiency seen in all patients, two had T-helper cell deficiency and two had T-suppressor cell hyperactivity. One of the latter patients was treated with cimetidine in an attempt to ablate histamine type 2 receptor-bearing suppressor cells: the absolute number of such cells was temporarily reduced but there was no concurrent correction of the functional hyperactivity. These studies point to the variable nature of T-regulatory cell deficiencies in hypogammaglobulinaemia.
authors.affiliation: Department of Pathology, Queen Mary Hospital, Hong Kong.
authors.forename: B M
authors.initials: BM
authors.lastname: Jones
authors.forename: Y L
authors.initials: YL
authors.lastname: Lau
authors.forename: K L
authors.initials: KL
authors.lastname: Wong
mesh_terms: D000293:Adolescent; D000361:Agammaglobulinemia / Q000188:drug therapy / Q000276:immunology*; D001402:B-Lymphocytes / Q000187:drug effects / Q000276:immunology*; D016516:CD4-CD8 Ratio; D002648:Child; D002675:Child, Preschool; D002681:China; D003208:Concanavalin A / Q000494:pharmacology / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D007963:Leukocytes, Mononuclear / Q000187:drug effects / Q000276:immunology; D008213:Lymphocyte Activation; D008297:Male; D016853:Muromonab-CD3 / Q000494:pharmacology / Q000627:therapeutic use; D011043:Pokeweed Mitogens / Q000494:pharmacology / Q000627:therapeutic use; D006377:T-Lymphocytes, Helper-Inducer / Q000187:drug effects / Q000276:immunology*; D050378:T-Lymphocytes, Regulatory / Q000187:drug effects / Q000276:immunology*
chemical_list: D016853:Muromonab-CD3; D011043:Pokeweed Mitogens; D003208:Concanavalin A