The first description of GATA2 mutation dates back to 2011, when four independent groups described four clinical phenotypes: Emberger syndrome (lymphedema and monosomy 7), MonoMAC syndrome (monocytopenia and Mycobacterium avium complex infection), dendritic cell, monocyte, B and natural killer (NK) lymphoid deficiency (DCML), and familial MDS/AML, all associated with a GATA2 deficiency [3,4,5]. The gene discussed is GATA2; the disease is Decreased total monocyte count.