Although the occurrence of HLH has been reported in a variety of PIDs (5, 6), the combination of GATA2-haploinsufficiency and HLH has, to the best of our knowledge, only been described twice in the context of herpes virus infection: once in a patient with herpes simplex virus infection (18), and once in a patient with marked EBV viremia and an EBV-driven T cell lymphoproliferative disease (19), but not with VZV. This evidence concerns the gene GATA2 and hemophagocytic syndrome.