Patients with germline GATA2–deficiency may present immunodeficiencies and myeloid neoplasms, including DCML deficiency (combined deficit of dendritic cell, monocyte, B and NK lymphoid cells), MonoMAC (monocytopenia and mycobacterial infection) with associated recurrent infections, myelodysplastic neoplasm (MDS), acute myeloid leukemia (AML), and chronic myelomonocytic leukemia [1, 2]. Here, GATA2 is linked to immunodeficiency disease.