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

  • Immunology/Allergology
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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.

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Abbreviations

CMV:

cytomegalovirus

Con A:

concanavalin A

HSV:

herpes simplex virus

PBMC:

peripheral blood mononuclear cells

PFC:

plaque-forming cell

PHA:

phytohaemagglutinin

PWM:

pokeweed mitogen

SAC:

Staphylococcus aureus Cowan I

WAS:

Wiskott-Aldrich syndrome

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Authors and Affiliations

  1. Department of Paediatrics, University of Hong Kong, Hong Kong

    Y. L. Lau, L. C. K. Low & S. N. Wong

  2. Department of Pathology, University of Hong Kong, Hong Kong

    B. M. Jones

  3. Paediatric Unit A, Princess Margaret Hospital, Hong Kong

    N. K. Leung

Authors
  1. Y. L. Lau
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  2. B. M. Jones
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  3. L. C. K. Low
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  4. S. N. Wong
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  5. N. K. Leung
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Lau, Y.L., Jones, B.M., Low, L.C.K. et al. Defective B-cell and regulatory T-cell function in Wiskott-Aldrich syndrome. Eur J Pediatr 151, 680–683 (1992). https://doi.org/10.1007/BF01957573

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  • DOI: https://doi.org/10.1007/BF01957573

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