These data suggest that EBV-specific immunity is impaired in BENTA patients, but pales in comparison to other PIDs such as X-linked lymphoproliferative syndrome (XLP) or MAGT1/CD27/CD70/ITK or Coronin1A deficiency diseases featuring exquisite susceptibility to severe EBV infection and disease (5, 20–22). This evidence concerns the gene CD27 and X-linked lymphoproliferative disease.