title: Defective B-cell and regulatory T-cell function in Wiskott-Aldrich syndrome.
abstract: We report two Chinese boys with Wiskott-Aldrich syndrome presenting with gastro-intestinal bleeding, eczema and recurrent infection. They had thrombocytopenia and the mean platelet volume was small. Serum IgG and IgA were elevated and lymphocyte proliferation in response to phytohaemagglutinin, concanavalin A and pokeweed mitogen was defective. Despite documented herpes simplex virus type 1 and cytomegalovirus infection in one patient, he did not mount any humoral response. The generation of antibody-secreting cells in response to pokeweed mitogen was markedly defective in a plaque-forming cell assay. Both patients' regulatory T-cell and B-cell functions were defective in this assay. The genetic defect in Wiskott-Aldrich syndrome therefore affects T-cells, B-cells and platelets.
authors.affiliation: Department of Paediatrics, University of Hong Kong.
authors.forename: Y L
authors.initials: YL
authors.lastname: Lau
authors.forename: B M
authors.initials: BM
authors.lastname: Jones
authors.forename: L C
authors.initials: LC
authors.lastname: Low
authors.forename: S N
authors.initials: SN
authors.lastname: Wong
authors.forename: N K
authors.initials: NK
authors.lastname: Leung
mesh_terms: D000917:Antibody Formation; D001402:B-Lymphocytes / Q000166:cytology / Q000502:physiology*; D002455:Cell Division; D006801:Humans; D007223:Infant; D008297:Male; D013601:T-Lymphocytes / Q000166:cytology / Q000502:physiology*; D014923:Wiskott-Aldrich Syndrome / Q000235:genetics / Q000276:immunology* / Q000473:pathology