Profound reduction of invariant natural killer T cells in the peripheral blood of a patient with interleukin-1 receptor-associated kinase 4 deficiency.

We previously described a patient with a history of frequent life-threatening pneumonias, infections with bacterial pathogens, interleukin 1 receptor-associated kinase 4 deficiency, and failure to maintain antibody titers to polysaccharide antigens or to a neoantigen bacteriophage. In the present study, we show that the patient's peripheral blood mononuclear cells have a profound deficiency of invariant natural killer T cells.