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

Chiara Messina, Dahlia Kirkpatrick, Patricia A. Fitzgerald, Richard J. O'Reilly, Frederick P. Siegal, Charlotte Cunningham-Rundles, Michael Blaese, James Oleske, Savita Pahwa, Carlos Lopez

Research output: Contribution to journalArticlepeer-review

27 Scopus citations


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-α 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-α 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-α 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.

Original languageEnglish (US)
Pages (from-to)394-404
Number of pages11
JournalClinical Immunology and Immunopathology
Issue number3
StatePublished - Jun 1986
Externally publishedYes

ASJC Scopus subject areas

  • Immunology and Allergy
  • Pathology and Forensic Medicine
  • Immunology


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