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Severe combined immunodeficiency with natural killer-cell predominance: abrogation of graft-versus-host disease and immunologic reconstitution with HLA-identical bone marrow cells.

Publication ,  Journal Article
Sindel, LJ; Buckley, RH; Schiff, SE; Ward, FE; Mickey, GH; Huang, AT; Naspitz, C; Koren, H
Published in: J Allergy Clin Immunol
June 1984

A 3 1/2-month-old infant with severe combined immunodeficiency was found to have an unusual blood lymphocyte phenotype. Thirty percent of her cells formed rosettes with sheep erythrocytes, but only 7.9% reacted with the pan T monoclonal antibody OKT3, and 5% reacted with an antibody (OKT4)-recognizing T-helper cells. Surprisingly 19.4% of her cells reacted with an antibody (OKT8)-recognizing T-suppressor cells and 94% reacted with OKT10 . Few reacted with other monoclonal antibodies detecting cellular activation antigens. Despite absence of T or B cell function, her monocyte-depleted blood lymphocytes caused a high degree of specific lysis of 51Cr-labeled K562 erythromyeloid cells in a natural killer-cell assay. Most of her lymphocytes were large and had azurophilic granules and a monocytoid nucleus. Because she had received a nonirradiated, unrelated red-cell transfusion 3 days earlier, 4.8 X 10(9) nucleated bone-marrow cells from her HLA-identical brother were given shortly after admission. Two days later a graft-versus-host reaction began but subsided completely within 3 days. On day 15 posttransplantation, a profuse secretory diarrhea began, accompanied by a rise in her serum IgE from 4 to 3000 IU. With engraftment, the number of T10+ cells and natural killer-cell function fell to normal, and full immunologic reconstitution was achieved.

Duke Scholars

Published In

J Allergy Clin Immunol

DOI

ISSN

0091-6749

Publication Date

June 1984

Volume

73

Issue

6

Start / End Page

829 / 836

Location

United States

Related Subject Headings

  • T-Lymphocytes, Regulatory
  • T-Lymphocytes
  • Phenotype
  • Lymphocyte Activation
  • Killer Cells, Natural
  • Karyotyping
  • Infant
  • Immunologic Deficiency Syndromes
  • Immunoglobulin E
  • Humans
 

Citation

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ICMJE
MLA
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Sindel, L. J., Buckley, R. H., Schiff, S. E., Ward, F. E., Mickey, G. H., Huang, A. T., … Koren, H. (1984). Severe combined immunodeficiency with natural killer-cell predominance: abrogation of graft-versus-host disease and immunologic reconstitution with HLA-identical bone marrow cells. J Allergy Clin Immunol, 73(6), 829–836. https://doi.org/10.1016/0091-6749(84)90455-x
Sindel, L. J., R. H. Buckley, S. E. Schiff, F. E. Ward, G. H. Mickey, A. T. Huang, C. Naspitz, and H. Koren. “Severe combined immunodeficiency with natural killer-cell predominance: abrogation of graft-versus-host disease and immunologic reconstitution with HLA-identical bone marrow cells.J Allergy Clin Immunol 73, no. 6 (June 1984): 829–36. https://doi.org/10.1016/0091-6749(84)90455-x.
Sindel LJ, Buckley RH, Schiff SE, Ward FE, Mickey GH, Huang AT, et al. Severe combined immunodeficiency with natural killer-cell predominance: abrogation of graft-versus-host disease and immunologic reconstitution with HLA-identical bone marrow cells. J Allergy Clin Immunol. 1984 Jun;73(6):829–36.
Sindel, L. J., et al. “Severe combined immunodeficiency with natural killer-cell predominance: abrogation of graft-versus-host disease and immunologic reconstitution with HLA-identical bone marrow cells.J Allergy Clin Immunol, vol. 73, no. 6, June 1984, pp. 829–36. Pubmed, doi:10.1016/0091-6749(84)90455-x.
Sindel LJ, Buckley RH, Schiff SE, Ward FE, Mickey GH, Huang AT, Naspitz C, Koren H. Severe combined immunodeficiency with natural killer-cell predominance: abrogation of graft-versus-host disease and immunologic reconstitution with HLA-identical bone marrow cells. J Allergy Clin Immunol. 1984 Jun;73(6):829–836.
Journal cover image

Published In

J Allergy Clin Immunol

DOI

ISSN

0091-6749

Publication Date

June 1984

Volume

73

Issue

6

Start / End Page

829 / 836

Location

United States

Related Subject Headings

  • T-Lymphocytes, Regulatory
  • T-Lymphocytes
  • Phenotype
  • Lymphocyte Activation
  • Killer Cells, Natural
  • Karyotyping
  • Infant
  • Immunologic Deficiency Syndromes
  • Immunoglobulin E
  • Humans