Lirilumab, a fully human IgG4 anti-KIR2D monoclonal antibody that recognizes the same epitope as IPH2101 and in a similar manner blocks the KIR interaction with HLA-C, has been well-tolerated in early phase clinical trials (99, 100) and has shown some clinical activity when combined with azacytidine in high risk myelodysplastic syndrome (MDS) (101). This evidence concerns the gene HLA-C and myelodysplastic syndrome.