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Pulmonary alveolar proteinosis
Marie-Rose Akin1, Gia-Khanh Nguyen
1Department of Pathology, St. Agnes Medical Center, Fresno, CA, USA.
Pathology, Research and Practice
|January 15, 2005
Summary
Pulmonary alveolar proteinosis (PAP) was diagnosed in three patients presenting with respiratory symptoms. Electron microscopy confirmed PAP, while HIV-positive patients also showed Pneumocystis carinii cysts.
Area of Science:
- Pulmonology
- Infectious Diseases
- Radiology
Background:
- Pulmonary alveolar proteinosis (PAP) is a rare lung disorder characterized by the accumulation of surfactant-associated material in the alveoli.
- Differentiating PAP from other diffuse alveolar diseases can be challenging, especially in immunocompromised patients.
Observation:
- Three adult patients, two with HIV, presented with acute dyspnea and dry cough.
- High-resolution CT scans showed a characteristic 'crazy-paving' pattern, suggesting PAP.
- Bronchoalveolar lavage (BAL) revealed proteinaceous material consistent with PAP.
Findings:
- Periodic acid-Schiff (PAS) staining confirmed the proteinaceous nature of the alveolar material.
- Pneumocystis carinii (PC) cysts were identified in BAL from HIV-positive patients.
- Electron microscopy confirmed numerous myelin figures, diagnostic of PAP in all cases.
Implications:
- This case series highlights the utility of high-resolution CT and BAL analysis in diagnosing PAP.
- The co-occurrence of PAP and Pneumocystis pneumonia in HIV-positive individuals underscores the importance of comprehensive diagnostic workup.
- Early and accurate diagnosis of PAP is crucial for appropriate management and improved patient outcomes.