SURGICAL PATHOLOGY OF THE LUNG IN CHRONIC GRANULOMATOUS-DISEASE

The pathologic features in pulmonary specimens are reported from 32 op en thoracotomies of 20 patients with chronic granulomatous disease (CG D). The pattern of inflammation present in the resected material varie d, but a granulomatous component was present in each case. In 78% of t he specimens, a distinctive form of granuloma was found: a neutrophili c microabscess surrounded by palisading histiocytes. In four specimens eosinophils were also found within the microabscesses. This feature w as found exclusively in cases of fungal infection. Fungal organisms we re found by culture in 18 specimens (56%) and in 17 of these specimens (94%), they also were seen by histopathology. In 9 cases (28%) routin e bacterial cultures were positive, and in one case an atypical Mycoba cterium was cultured. These organisms were not prospectively identifie d on special stains of histologic sections in any of the cases. Absces s formation was found more commonly in pure fungal infections (41%) th an in pure bacterial infections (14%). In contrast to earlier reports, well-formed granulomas with giant cells were not specific for fungal infections. In this series, they were present in 57% of cases with pur e bacterial infections. A subset of the patients received gamma interf eron therapy or granulocyte transfusions before the surgical procedure s. No differences in the histopathology of the inflammation were assoc iated with granulocyte transfusions, but gamma interferon therapy,vas associated with a reduction in necrotizing granulomatous inflammation. Additionally, one case of severe cytomegalovirus pneumonitis is descr ibed in a CGD patient receiving chronic steroid therapy.