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Primary immunodeficiency diseases: dissectors of the immune system.

Publication ,  Journal Article
Buckley, RH
Published in: Immunol Rev
July 2002

The past 50 years have seen enormous progress in this field. An unknown concept until 1952, there are now more than 100 different primary immunodeficiency syndromes in the world's literature. Each novel syndrome has shed new insight into the workings of the immune system, dissecting its multiple parts into unique functioning components. This has been especially true over the past decade, as the molecular bases of approximately 40 of these diseases have been identified in rapid succession. Advances in the treatment of these diseases have also been impressive. Antibody replacement has been improved greatly by the development of human immunoglobulin preparations that can be safely administered by the intravenous route, and cytokine and humanized anticytokine therapies are now possible through recombinant technologies. The ability to achieve life-saving immune reconstitution of patients with lethal severe combined immunodeficiency by administering rigorously T-cell-depleted allogeneic related haploidentical bone marrow stem cells has extended this option to virtually all such infants, if diagnosed before untreatable infections develop. Finally, the past 3 years have witnessed the first truly successful gene therapy. The impressive results in X-linked severe combined immunodeficiency offer hope that this approach can be extended to many more diseases in the future.

Duke Scholars

Published In

Immunol Rev

DOI

ISSN

0105-2896

Publication Date

July 2002

Volume

185

Start / End Page

206 / 219

Location

England

Related Subject Headings

  • Severe Combined Immunodeficiency
  • Male
  • Job Syndrome
  • Immunology
  • Immunologic Deficiency Syndromes
  • Immune System
  • Humans
  • History, 20th Century
  • Genetic Therapy
  • Female
 

Citation

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Buckley, R. H. (2002). Primary immunodeficiency diseases: dissectors of the immune system. Immunol Rev, 185, 206–219. https://doi.org/10.1034/j.1600-065x.2002.18517.x
Buckley, Rebecca H. “Primary immunodeficiency diseases: dissectors of the immune system.Immunol Rev 185 (July 2002): 206–19. https://doi.org/10.1034/j.1600-065x.2002.18517.x.
Buckley RH. Primary immunodeficiency diseases: dissectors of the immune system. Immunol Rev. 2002 Jul;185:206–19.
Buckley, Rebecca H. “Primary immunodeficiency diseases: dissectors of the immune system.Immunol Rev, vol. 185, July 2002, pp. 206–19. Pubmed, doi:10.1034/j.1600-065x.2002.18517.x.
Buckley RH. Primary immunodeficiency diseases: dissectors of the immune system. Immunol Rev. 2002 Jul;185:206–219.

Published In

Immunol Rev

DOI

ISSN

0105-2896

Publication Date

July 2002

Volume

185

Start / End Page

206 / 219

Location

England

Related Subject Headings

  • Severe Combined Immunodeficiency
  • Male
  • Job Syndrome
  • Immunology
  • Immunologic Deficiency Syndromes
  • Immune System
  • Humans
  • History, 20th Century
  • Genetic Therapy
  • Female