Deficiency of inducible suppressor cell activity in the Chediak‐Higashi syndrome

Peripheral blood lymphocytes from two Chediak‐Higashi syndrome (CHS) patients were examined for their 1) natural killer (NK) cell functions 2) concanavalin A (Con A)‐inducible suppressor cell activity, 3) soluble suppressor factor production, and 4) responsiveness to interferon alpha and interleukin‐2 in comparison with age‐matched normal controls. Peripheral blood lymphocytes or NK‐enriched large granular lymphocytes from Chediak‐Higashi syndrome patients showed negligible cytotoxic activity against several target cells. Although the NK activity of Chediak‐Higashi syndrome lymphocytes could not be restored to normal levels by treatment with either interferon or interleukin‐2, the percent enhancement of NK activity was higher for the patients than the controls. Soluble suppressor factor activity of culture supernates from the lymphocytes of Chediak‐Higashi syndrome patients significantly inhibited the NK activity of allogeneic, normal peripheral blood lymphocytes, whereas lymphocytes from Chediak‐Higashi syndrome patients precultured with Con A failed to suppress the cytotoxic activity of normal lymphocytes. These results demonstrate a previously unrecognized suppressor cell dysfunction in CHS patients.