CTICasesGATA2 Deficiency with Follicular Bronchiolitis and Cystic Lung Destruction
Imaging findings
CT demonstrates upper-lobe-predominant, bizarre cystic spaces adjacent to airways with centrilobular nodules superimposed on a background of upper lobe lung destruction, in a patient with GATA2 deficiency.
Key takeaways
GATA2 deficiency is a rare inherited immunodeficiency associated with lymphedema, myelodysplasia, and pulmonary alveolar proteinosis. Pulmonary manifestations include recurrent opportunistic infections and follicular bronchiolitis, which can progress to cystic parenchymal destruction, particularly in the upper lobes, resembling combined findings not typical of common diagnoses.
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