Haploinsufficiency of GATA2 underlies five distinct syndromes: (1) Mono-cytopenia and non-tuberculous mycobacterial infection (MonoMAC); (2) Dendritic cell, monocyte, B, and natural killer lymphopenia (DCML); (3) Familial myelodysplasia (MDS)/acute myelogenous leukemia (AML); (4) Emberger syndrome (primary lymphedema with MDS); and (5) classical NK cell deficiency1–3. This evidence concerns the gene GATA2 and lymphedema.