Expression of KIR2DL1 on the entire NK cell population: a possible novel immunodeficiency syndrome Academic Article uri icon


  • The killer immunoglobulin-like receptor (KIR) family recognizes class I major histocompatibility complex (MHC) molecules and inhibits natural killer (NK) activity. This family is highly polymorphic, and only some of the genes are present in each individual. 1 Thus, the frequency of a single inhibitory receptor in a bulk NK population varies between 5% and 50%. 2 It was suggested that such diversity of NK receptors enables the killing of infected cells by at least a subset of NK cells. 3 A 7-year-old patient from Israel had been hospitalized 12 times due to infections. At the age of 2.5 months, the patient had severe interstitial pneumonitis, hepatosplenomegaly, anemia, and thrombocytopenia. Immunoglobulin M (IgM) antibodies against cytomegalovirus (CMV) were identified, and CMV in the urine was detected. At the age of 2.5 years, the patient had a severe bilateral …

publication date

  • January 1, 2004

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