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Presented by Dr. Cimino-Mathews and prepared by Dr. Armen Khararjian
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45 year-old female presents with shortness of breath
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1. Question
Week 632: Case 1
45 year-old female presents with shortness of breath
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Answer: D. Pneumocystis pneumonia
Histology: The open lung biopsy reveals alveolated lung parenchyma with thickened alveolar walls with interstitial chronic inflammatory cells, carbon pigment, reactive pneumocyte hyperplasia and “fibroblast foci” or plugs that are characteristic of “bronchiolitis obliterans organizing pneumonia (BOOP)” or “cytotogenic organizing pneumonia”. The injury pattern is diffuse. There are no granulomas or abscesses. The alveolar spaces are filled with pink exudate that has a heterogenous, foamy appearance. A Grocott’s methenamine silver (GMS) stain highlights small, cup-shaped and round yeast-like cyst forms within the alveolar exudate.
Discussion: The histologic features and special stain results are diagnostic of Pneumocystis pneumonia, caused by the fungus Pneumocystis jiroveci (formerly Pneumocystis carinii). Pneumocystis pneumonia is an AIDS-defining illness in HIV infected individuals and is in fact the most common opportunistic infection in HIV infected individuals. Pneumocystis jiroveci cannot be cultured, so the diagnosis must be made histologically on induced sputum samples or bronchioalveolar lavage samples, but if those specimens are nondiagnostic, a transbronchial or open lung biopsy is the most sensitive method of detection. The classic histologic feature is the presence of alveolar exudates with pink “frothy” material, and Pneumocystis should always be excluded in that histology picture. The cyst forms can be detected with special stains such as GMS, or a Pneumocystis jiroveci-specific immunostain. The cyst forms are often described as “cup shaped” or like “ping pong balls” and are approximately half the size of red blood cells (which are typically 8 microns and a useful internal control for size). Other histologic features that may be present include fibroblast foci characteristic of BOOP, as well as rarely granulomatous inflammation. Pneumocystis infection can also occur concomitantly with viral infection, particularly since the patients are typically immunocompromised, so histologic examination for viral inclusions is also important.
References
1. Gordon IO1, Cipriani N, Arif Q, Mackinnon AC, Husain AN. Update in nonneoplastic lung diseases. Arch Pathol Lab Med. 2009 Jul;133(7):1096-105.
2. Thomas CF, Limper AH. Pneumocystis Pneumonia. N Engl J Med 2004; 350:2487-2498.
3. Wazir JF, Ansari NA. Pneumocystis carinii infection. Update and review. Arch Pathol Lab Med. 2004 Sep;128(9):1023-7.Incorrect
Answer: D. Pneumocystis pneumonia
Histology: The open lung biopsy reveals alveolated lung parenchyma with thickened alveolar walls with interstitial chronic inflammatory cells, carbon pigment, reactive pneumocyte hyperplasia and “fibroblast foci” or plugs that are characteristic of “bronchiolitis obliterans organizing pneumonia (BOOP)” or “cytotogenic organizing pneumonia”. The injury pattern is diffuse. There are no granulomas or abscesses. The alveolar spaces are filled with pink exudate that has a heterogenous, foamy appearance. A Grocott’s methenamine silver (GMS) stain highlights small, cup-shaped and round yeast-like cyst forms within the alveolar exudate.
Discussion: The histologic features and special stain results are diagnostic of Pneumocystis pneumonia, caused by the fungus Pneumocystis jiroveci (formerly Pneumocystis carinii). Pneumocystis pneumonia is an AIDS-defining illness in HIV infected individuals and is in fact the most common opportunistic infection in HIV infected individuals. Pneumocystis jiroveci cannot be cultured, so the diagnosis must be made histologically on induced sputum samples or bronchioalveolar lavage samples, but if those specimens are nondiagnostic, a transbronchial or open lung biopsy is the most sensitive method of detection. The classic histologic feature is the presence of alveolar exudates with pink “frothy” material, and Pneumocystis should always be excluded in that histology picture. The cyst forms can be detected with special stains such as GMS, or a Pneumocystis jiroveci-specific immunostain. The cyst forms are often described as “cup shaped” or like “ping pong balls” and are approximately half the size of red blood cells (which are typically 8 microns and a useful internal control for size). Other histologic features that may be present include fibroblast foci characteristic of BOOP, as well as rarely granulomatous inflammation. Pneumocystis infection can also occur concomitantly with viral infection, particularly since the patients are typically immunocompromised, so histologic examination for viral inclusions is also important.
References
1. Gordon IO1, Cipriani N, Arif Q, Mackinnon AC, Husain AN. Update in nonneoplastic lung diseases. Arch Pathol Lab Med. 2009 Jul;133(7):1096-105.
2. Thomas CF, Limper AH. Pneumocystis Pneumonia. N Engl J Med 2004; 350:2487-2498.
3. Wazir JF, Ansari NA. Pneumocystis carinii infection. Update and review. Arch Pathol Lab Med. 2004 Sep;128(9):1023-7.
