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. 1982 Jul;46(3):555–560.

Further studies of natural killer cell function in Chediak-Higashi patients.

J C Roder, T Haliotis, L Laing, D Kozbor, P Rubin, H Pross, L A Boxer, J G White, A S Fauci, H Mostowski, D S Matheson
PMCID: PMC1555330  PMID: 6212535

Abstract

Spontaneous natural killer (NK) activity and antibody-dependent cellular cytotoxicity (ADCC) of blood lymphocytes against five human tumour cell lines (K562, Molt-4, HL-60, Chang, Daudi) and three mouse tumour lines (YAC, P815, RBL-5) were ten- to 100-fold lower than normal in six patients with Chediak-Higashi (CH) disease. NK and ADCC were defective at 4 hr, and less so at 18 hr. 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. These observations confirm that the CH syndrome is associated with a profound and selective defect in NK and ADCC activity against tumour cells, whereas other mononuclear cell-mediated functions are normal.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Blume R. S., Wolff S. M. The Chediak-Higashi syndrome: studies in four patients and a review of the literature. Medicine (Baltimore) 1972 Jul;51(4):247–280. [PubMed] [Google Scholar]
  2. Dent P. B., Fish L. A., White L. G., Good R. A. Chediak-Higashi syndrome. Observations on the nature of the associated malignancy. Lab Invest. 1966 Oct;15(10):1634–1642. [PubMed] [Google Scholar]
  3. Gallin J. I., Elin R. J., Hubert R. T., Fauci A. S., Kaliner M. A., Wolff S. M. Efficacy of ascorbic acid in Chediak-Higashi syndrome (CHS): studies in humans and mice. Blood. 1979 Feb;53(2):226–234. [PubMed] [Google Scholar]
  4. Griscelli C., Durandy A., Guy-Grand D., Daguillard F., Herzog C., Prunieras M. A syndrome associating partial albinism and immunodeficiency. Am J Med. 1978 Oct;65(4):691–702. doi: 10.1016/0002-9343(78)90858-6. [DOI] [PubMed] [Google Scholar]
  5. Nelson D. L., Bundy B. M., Pitchon H. E., Blaese R. M., Strober W. The effector cells in human peripheral blood mediating mitogen-induced cellular cytotoxicity and antibody-dependent cellular cytotoxicity. J Immunol. 1976 Nov;117(5 Pt 1):1472–1481. [PubMed] [Google Scholar]
  6. Pross H. F., Baines M. G., Rubin P., Shragge P., Patterson M. S. Spontaneous human lymphocyte-mediated cytotoxicity against tumor target cells. IX. The quantitation of natural killer cell activity. J Clin Immunol. 1981 Jan;1(1):51–63. doi: 10.1007/BF00915477. [DOI] [PubMed] [Google Scholar]
  7. Roder J. C., Haliotis T., Klein M., Korec S., Jett J. R., Ortaldo J., Heberman R. B., Katz P., Fauci A. S. A new immunodeficiency disorder in humans involving NK cells. Nature. 1980 Apr 10;284(5756):553–555. doi: 10.1038/284553a0. [DOI] [PubMed] [Google Scholar]
  8. Roder J., Duwe A. The beige mutation in the mouse selectively impairs natural killer cell function. Nature. 1979 Mar 29;278(5703):451–453. doi: 10.1038/278451a0. [DOI] [PubMed] [Google Scholar]
  9. Sullivan J. L., Byron K. S., Brewster F. E., Purtilo D. T. Deficient natural killer cell activity in x-linked lymphoproliferative syndrome. Science. 1980 Oct 31;210(4469):543–545. doi: 10.1126/science.6158759. [DOI] [PubMed] [Google Scholar]

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