In the past decade, GATA2 deficiency has been recognized to cause previously distinct clinical entities, namely DCML deficiency (dendritic cell, monocyte, B, and NK lymphoid deficiency) (2), MonoMAC syndrome (monocytopenia with Mycobacterium avium complex infection) (3), familial myelodysplastic syndrome (MDS)/acute myeloid leukemia (AML) (4), Emberger syndrome (MDS with lymphedema) (5), and NK deficiency (6, 7). The gene discussed is GATA2; the disease is Decreased total monocyte count.