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Chronic Granulomatous Disease, Kx Negative Neutrophils and Linkage with Xg

  • Peter Densen
  • Susan Wilkinson
  • Gerald L. Mandell
  • Gail Sullivan
  • Ragnhild Øyen
  • W. L. Marsh
  • R. A. Harkness
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 141)

Abstract

Individuals afflicted with chronic granulomatous disease (CGD) suffer from recurrent infections due to the inability of their neutrophils to kill ingested microorganisms. Defective microbicidal function is a consequence of the inability of these cells to generate metabolites of oxygen which are toxic to ingested bacteria. This defect in oxidative activity is believed to be due to the absence or dysfunction of a membrane bound oxidase. In 1975, Marsh and co-workers (1) reported that neutrophils from patients with X-linked CGD, but not other forms of CGD, lacked a surface antigen called Kx. Kx is an X-linked, Kell-related antigen normally present on the surface of both red cells and granulocytic white cells. Its distribution on these two cell types is determined by any of four alleles that have been described at the Xk locus (2). Erythrocytic Kx is converted to the appropriate Kell antigen by an autosomally determined enzyme. Thus, Kx is believed to serve as a precursor substance for Kell antigens on red cells, whereas on granulocytes it remains unaltered.

Keywords

Chronic Granulomatous Disease Normal Neutrophil Infectious Disease Department Hemagglutination Reaction Clinical Research Department 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

  1. 1.
    W.L. Marsh, R. Oyen, M.E. Nichols, and F.H. Allen, Chronic granulomatous disease and the Kell blood groups, Brit. J. Haematol. 29: 247 (1975).CrossRefGoogle Scholar
  2. 2.
    W.L. Marsh, R. Oyen, and M.E. Nichols, Kx antigen, the McLeod phenotype and chronic granulomatous disease: Fur ther studies, Vox. Sang. 31: 356 (1976).PubMedCrossRefGoogle Scholar
  3. 3.
    J.A. Dilworth and G.L. Mandell, Adults with chronic granuloma-tous disease of “childhood,” Amer. J. Med. 63: 233 (1977).PubMedCrossRefGoogle Scholar
  4. 4.
    Technical Methods and Procedures, Amer. Assn. Blood Banks, 7th ed., Washington, D.C. (1977).Google Scholar
  5. 5.
    W.L. Marsh, Scoring of hemagglutination reactions, Transfusion 12: 352 (1972).PubMedCrossRefGoogle Scholar

Copyright information

© Plenum Press, New York 1982

Authors and Affiliations

  • Peter Densen
    • 1
    • 2
    • 3
  • Susan Wilkinson
    • 1
    • 2
    • 3
  • Gerald L. Mandell
    • 1
    • 2
    • 3
  • Gail Sullivan
    • 1
    • 2
    • 3
  • Ragnhild Øyen
    • 1
    • 2
    • 3
  • W. L. Marsh
    • 1
    • 2
    • 3
  • R. A. Harkness
  1. 1.Evans Memorial Department of Clinical Research Department of MedicineBoston University Medical CenterBostonUSA
  2. 2.Division of Infectious Diseases Departments of Internal Medicine and PathologyUniversity of VirginiaCharlottesvilleUSA
  3. 3.The Lindsley F. Kimball Research Institute of The New York Blood CenterNew YorkUSA

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