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

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, conc...

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Bibliographic Details
Published in:European journal of pediatrics 1992-09, Vol.151 (9), p.680-683
Main Authors: LAU, Y. L, JONES, B. M, LOW, L. C. K, WONG, S. N, LEUNG, N. K
Format: Article
Language:English
Subjects:
Antibody Formation
B-Lymphocytes - cytology
B-Lymphocytes - physiology
Biological and medical sciences
Cell Division
Hematologic and hematopoietic diseases
Humans
Infant
Male
Medical sciences
Platelet diseases and coagulopathies
T-Lymphocytes - cytology
T-Lymphocytes - physiology
Wiskott-Aldrich Syndrome - genetics
Wiskott-Aldrich Syndrome - immunology
Wiskott-Aldrich Syndrome - pathology
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Summary: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.
ISSN:0340-6199
1432-1076
DOI:10.1007/BF01957573