DCs deficiency (in both myeloid and plasmocytoid subsets) has been reported in patients with heterozygous germline GATA2 mutations, a heterogeneous disorder characterized by familial myelodysplasia/acute myeloid leukemia (MSD/AML), the MonoMac (monocytopenia with Mycobacterium avium complex) syndrome, and/or the Emberger syndrome (primary lymphedema and MSD/AML) (17). The gene discussed is GATA2; the disease is primary lymphedema.