Pulmonary Alveolar Proteinosis and Pulmonary Hypertension

 

Pulmonary alveolar proteinosis and pulmonary hypertension occurs in approximately one-third of GATA2 deficiency patients.  These pulmonary changes seen in this disease likely reflect pulmonary alveolar macrophage dysfunction and defective transcriptional regulation of endothelial nitric oxide synthetase.   

GATA2 Deficiency

PULMONARY ALVEOLAR PROTEINOSIS AND PULMONARY HYPERTENSION