Abnormalities of Chemotactic Lymphokine Synthesis and Mononuclear Leukocyte Chemotaxis in Wiskott-Aldrich Syndrome

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Abstract

Wiskott-Aldrich syndrome is characterized by numerous humoral and cellular immune abnormalities including anergy, defective antibody production, and increased immunoglobulin synthesis. To define better the mechanisms of defective cellular immunity in this disorder, lymphoproliferative responses, lymphokine production, and the chemotactic responsiveness of mononuclear leukocytes (MNL) from patients with Wiskott-Aldrich syndrome were quantitated. Peripheral blood lymphocytes from these patients produced normal amounts of a lymphocyte-derived chemotactic factor (LDCF); however, their lymphoproliferative responses were frequently depressed, particularly to antigenic stimuli. In the absence of exogenous antigens or mitogens, lymphocytes from patients with Wiskott-Aldrich syndrome produced significantly more LDCF than unstimulated normal lymphocytes. In fact, this unstimulated LDCF production frequently approached the level produced by normal cells only after antigen or mitogen stimulation.

Authors

Leonard C. Altman, Ralph Snyderman, R. Michael Blaese