FURTHER-STUDIES OF NATURAL-KILLER CELL-FUNCTION IN CHEDIAK-HIGASHI PATIENTS

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  • 1 January 1982
    • journal article
    • research article
    • Vol. 46  (3) , 555-560
Abstract
Spontaneous natural killer (NK) activity and antibody-dependent cellular cytotoxicity (ADCC) of blood lymphocytes against 5 human tumor cell lines (K562[myeloid leukemia cells], Molt-4 [lymphocytic leukemia T-cells], H1-60 [promyelocytic myelogenous leukemia cells], Chang [hepatocarcinoma cells] Daudi [B-lymphoblastoid cells]) and 3 mouse tumor lines (YAC [T-cell lymphoma], P815 [mastocytoma cells], RBL-5 [lymphoma cells]) were 10- to 100-fold lower than normal in 6 patients with Chediak-Higashi (CH) disease. NK and ADCC were defective at 4 h and less so at 18 h. The NK activity in normals and CH patients was mediated in part by FcR+, E- effector cells. ADCC against human erythrocytes was normal in CH patients, as were lectin-dependent cytolysis and mixed lymphocyte proliferative responses. Phagocytosis of antibody-coated ox erythrocytes was normal in CH patients as well. The CH syndrome is evidently associated with a profound and selective defect in NK and ADCC activity against tumor cells, while other mononuclear cell-mediated functions are normal.