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Studies on hyperimmunoglobulinemia E syndrome in five children.

T cell subpopulations, lymphoproliferative response, autologous mixed lymphocyte reaction (AMLR), antibody-dependent cellular cytotoxicity (ADCC), natural killer (NK) activity and chemotaxis were studied in five hyperimmunoglobulinemia E (hyper IgE) syndrome patients. Both absolute OKT8 (suppressor T) cells and OKIa1 cells were decreased in number. The OKT4/OKT8 ratio increased. The lymphoproliferative response showed that the patients' lymphocytes cultured in medium containing autologous plasma proliferated much less vigorously, as expressed by transformation index, than those cultured in AB healthy control plasma. The peak proliferative response in AMLR in patients was significantly lower than that in the control group. The proliferative response of the fresh autologous mononuclear cells (MNC) exerted suppressor activity in the patients, while those from the normal controls exerted helper activity. Supernatant from AMLR and an isolated T cell culture of patients exerted suppressor activity, while those from the normal controls had none. In the ADCC reaction, patient's polymorphonuclears were significantly impaired in target cell lysis. The NK activity and Leu-11 cells in the patients also showed a significant decrease. In vitro neutrophil chemotaxis was consistently impaired in two out of five patients. Therefore, there is evidence of abnormal regulatory T cell functions and defect in PMN-ADCC activity and NK lysis assay in patients with hyper IgE syndrome.