Typical syndromes related to GATA2-haploinsufficiency include primary lymphoedema with myelodysplasia (Emberger syndrome), monocytopenia and mycobacterial infection (MonoMAC) syndrome, and dendritic cell, monocyte, B and NK lymphoid (DCML) deficiency (2). This evidence concerns the gene GATA2 and Decreased total monocyte count.