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.