Granulomatous Aspergillosis

Chronic granulomatous disease: Although a granulomatous response may occur with tracheobronchial and chronic necrotizing aspergillosis, a different type of granulomatous response to the organism is typically seen in patients with chronic granulomatous disease (CGD), an inherited condition characterized by susceptibility to certain bacterial and fungal infections as a result of defective phagocytes [1,2]. Aspergillus is the most common fungal pathogen in these patients, and it causes two forms of pneumonia, depending, in part, on the dose of organisms. In one form, there are one or more radiographic consolidations. In the other, which occurs after exposure to massive numbers of conidia, there is a miliary or reticulonodular infiltrate. In both types, histologic sections show purulent granulomas, either confluent or discrete [3-6]. Eosinophils may occasionally be present [6].

Histologic changes: This granuloma (between arrows) has a rim of epithelioid cells surrounding a purulent center of PMNs. A Gomori methenamine silver stain (not shown) showed a few organisms in the purulent center but not elsewhere. Similar granulomas were scattered diffusely throughout both lungs and had become confluent in some areas. This person with known CGD had inhaled of a massive dose of conidia while shoveling moldy wood chips. Death occurred 17 days following the exposure [5].

This pattern of response to aspergillus should suggest the diagnosis of CGD even in adults in whom the diagnosis has not yet been made [4].

Therapy for these patients includes prophylactic antibiotics, gamma interferon [1], bone marrow transplantation [7], and gene replacement [8].

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References:

1. Babior B, Woodman R. Chronic granulomatous disease. Sem Hematol 1990; 27:247-259.

2. Schapiro B, Newburger P, Klempner M, Dinauer M. Chronic granulomatous disease presenting in a 69-year-old man. New Engl J Med 1991; 325:1786-1790.

3. Carson M, Chadwick D, Brubaker C, Cleland R, Landing B. Thirteen boys with progressive septic granulomatosis. Pediatrics 1965; 35:405-411.

4. Kelly J, Pinto A, Whitelaw W, Rorstad O, Bowen T, Matheson D. Fatal aspergillus pneumonia in chronic granulomatous disease. Am J Clin Pathol 1986; 86:235-240.

5. Conrad D, Warnock M, Blanc P, Cowan M, Golden J. Microgranulomatous aspergillosis after shoveling wood ships: report of a fatal outcome in a patient with chronic granulomatous disease. Am J Indust Med 1992; 22:411-418.

6. Moskaluk C, Pogrebniak H, Pass H, Gallin J, Travis W. Surgical pathology of the lung in chronic granulomatous disease. Am J Clin Pathol 1994; 102:684-691.

7. Ho C, Vowels M, Lockwood L, Ziegler J. Successful bone marrow transplantation in a child with X-linked chronic granulomatous disease. Bone Marrow Transplant 1996; 18:213-215.

8. Malech H, Bauer T Jr, Hickstein D. Prospects for gene therapy of neutrophil defects. Sem Hematol 1997; 34:355-361.

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