In addition to its hematologic manifestations, the spectrum of GATA2-related disease encompasses, among others, immunodeficiency with monocytopenia, B- and NK-cell lymphopenia, pulmonary disease, typically in the form of pulmonary alveolar proteinosis (PAP), as well as syndromic features like congenital deafness and lymphedema (5). The gene discussed is GATA2; the disease is pulmonary alveolar proteinosis.