Abstract
Lymphocytes (PBL) from 2 Chediak-Higashi (CH) syndrome patients were examined for natural killer (NK) activity, antibody dependent cellular cytotoxicity (ADCC), target binding capacity (TBC), lectin dependent cellular cytotoxicity (LDCC), Con A induced suppressor cells, soluble suppressor factor (SSF), and responsiveness to interferon (IF) and interleukin-2 (IL-2). PBL from CH patients showed little NK or ADCC activity at various effector to target cell ratios. However, the TBC of patients' PBL was comparable with controls, indicating that the defect in cytotoxicity was not due to lack of target recognition. PBL from CH patients cultured with and without Con A failed to suppress the NK activity of fresh allogeneic effector cells in contrast to normal PBL, suggesting that defects in immunoregulatory lymphocytes may coexist with abnormal effector functions in CH patients. Although residual cytotoxicity of CH lymphocytes could be considerably enhanced with PHA in the LDCC system, enhancement was markedly less than that of normal control cells. Preincubating CH lymphocytes for 24 hr produced a slight increase in NK activity when IF was added to the cultures, whereas IL-2 did not enhance cytotoxicity in contrast when normal PBL were precultured with IF and IL-2. SSF from CH patients significantly inhibited the NK activity of normal PBL. The diminished cytotoxicity, defective immunoregulatory functions and reduced responsiveness to IF, IL-2 and LDCC of PBL from CH patients may contribute to their increased risk of infection and malignancies.
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Nair, M., Schwartz, S. & Boxer, L. DEFECTIVE IMMUNOREGULATORY FUNCTION OF LYMPHOCYTES FROM PATIENTS WITH THE CHEDIAK-HIGASHI SYNDROME. Pediatr Res 18 (Suppl 4), 260 (1984). https://doi.org/10.1203/00006450-198404001-01002
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DOI: https://doi.org/10.1203/00006450-198404001-01002