GATA2 and Decreased total monocyte count: GATA2 deficiency can manifest with diverse immunological features, linking it to various clinical entities once believed to be separate and distinct; these include MonoMAC syndrome (monocytopenia with Mycobacterium avium complex infection), DCML deficiency (dendritic cell, monocyte, B-, and NK-cell deficiency) and Emberger syndrome (MDS with lymphedema) (24).