Natural killer cell function and interferon generation in patients with primary immunodeficiencies.

Abstract

Patients with primary immunodeficiency disorders were evaluated for three aspects of natural defense: natural killer (NK) cells which lyse HSV-infected fibroblasts [NK(HSV-FS)], NK cells which lyse K562 tumor targets [NK(K562)], and interferon-alpha generation. In addition, capacity to make interferon upon challenge with other commonly used inducers was also evaluated. Most patients with severe combined immunodeficiency disease (SCID) and deficits of both T- and B-cell function demonstrated normal NK function with one or both targets. Six of eight SCID patients generated interferon-alpha at or below the lower limit of normal while only two made clearly normal levels. Six of 10 patients with Wiskott-Aldrich syndrome (WAS) had normal NK(K562) and five of 10 generated normal levels of interferon-alpha but all had severely deficient NK(HSV-FS). Patients with Bruton's agammaglobulinemia demonstrated normal NK and interferon generation, as did patients with common variable immunodeficiency, even when subdivided into patients with T-cell proliferative deficiencies and those with only hypogammaglobulinemia. Natural defense parameters may help categorize patients with SCID and WAS and help define these heterogeneous diseases.

• Authors: C Messina, D Kirkpatrick, P A Fitzgerald, R J O'Reilly, F P Siegal, C Cunningham-Rundles, M Blaese, J Oleske, S Pahwa, C Lopez
• Journal: Clinical immunology and immunopathology (Clin Immunol Immunopathol) Vol. 39 Issue 3 Pg. 394-404 (Jun 1986) ISSN: 0090-1229 [Print] United States
• PMID: 3698344 (Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Interferon Type I

Topics

• Agammaglobulinemia (immunology)
• Humans
• Immunity, Cellular
• Immunity, Innate
• Immunologic Deficiency Syndromes (immunology)
• Interferon Type I (biosynthesis)
• Killer Cells, Natural (immunology)
• Wiskott-Aldrich Syndrome (immunology)