Four different phenotypes associated with a GATA2 deficiency have been described: (i) Emberger syndrome (lymphedema and monosomy 7); (ii) MonoMAC syndrome (Monocytopenia and Mycobacterium avium complex infection); (iii) dendritic cell, monocyte, B and natural killer (NK) lymphoid deficiency (DCML); and (iv) familial MDS/AML [33,34,35]. The gene discussed is GATA2; the disease is myelodysplastic syndrome.