Natural killer cell function and interferon generation in patients with primary immunodeficiencies.
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.
Messina
C
C
Kirkpatrick
D
D
Fitzgerald
P A
PA
O'Reilly
R J
RJ
Siegal
F P
FP
Cunningham-Rundles
C
C
Blaese
M
M
Oleske
J
J
Pahwa
S
S
Lopez
C
C
D000361:Agammaglobulinemia / Q000276:immunology; D006801:Humans; D007111:Immunity, Cellular; D007113:Immunity, Innate; D007153:Immunologic Deficiency Syndromes / Q000276:immunology*; D007370:Interferon Type I / Q000096:biosynthesis*; D007694:Killer Cells, Natural / Q000276:immunology*; D014923:Wiskott-Aldrich Syndrome / Q000276:immunology
D007370:Interferon Type I