Defective B-cell and regulatory T-cell function in Wiskott-Aldrich syndrome

Eur J Pediatr. 1992 Sep;151(9):680-3. doi: 10.1007/BF01957573.

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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antibody Formation
  • B-Lymphocytes / cytology
  • B-Lymphocytes / physiology*
  • Cell Division
  • Humans
  • Infant
  • Male
  • T-Lymphocytes / cytology
  • T-Lymphocytes / physiology*
  • Wiskott-Aldrich Syndrome / genetics
  • Wiskott-Aldrich Syndrome / immunology*
  • Wiskott-Aldrich Syndrome / pathology