Severe and selective deficiency of interferon-γ-producing invariant natural killer T cells in patients with myelodysplastic syndromes

Here we show that patients with myelodysplastic syndromes (MDS) have a severe deficiency of glycolipid reactive Valpha24(+) /Vbeta11(+) natural killer T (NKT) cells, but not NK cells or CD4(+) or CD8(+) T cells. Neither the blood nor marrow of MDS patients had detectable interferon-gamma-producing NKT cells in response to the NKT ligand, alpha-galactosyl ceramide, although influenza-virus-specific effector T-cell function was preserved. This severe and selective deficiency of an important immune regulatory cell may contribute to the pathogenesis of MDS.