Case 3 -

Human Parainfluenza Virus Three Pneumonia with Associated Alveolar Proteinosis Thomas A. Sporn Duke University School of Medicine Durham, NC

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Clinical History The patient was a 2 year old girl diagnosed at 18 months of age with acute monocytic leukemia ( French-American-British M5). Following establishment of remission with standard chemotherapy and subsequent intensification and consolidation phases, the child suffered bone marrow relapse, which was treated with non-related umbilical cord blood transplant. The child developed progressive pulmonary infiltrates and alveolar opacities with hypoxic respiratory failure and death on post-transplant day 36, despite therapies directed against graft-versus-host disease, and bacterial, fungal and viral pathogens. An autopsy was performed, with findings notable for heavy lungs whose cut surface exuded pale yellow fluid. Microscopic examination of the lungs demonstrated alveolar filling with granular eosinophilic PAS-positive material. The lung parenchyma featured scant interstitial inflammation and fibrosis, and numerous syncytial-type giant cells. Alveolar epithelial differentiation for these cells was suggested on the basis of positive immunostaining with anti-cytokeratin antibodies. Immunofluorescent staining of the giant cells with human parainfluenza virus three (HPIV-3) was positive, and viral cultures bronchoalveolar lavage fluid obtained before death were also positive for HPIV-3. Ultrastructural examination of the alveolar exudate was notable for electron-dense granular material, cell debris and lamellar bodies with closely packed circumscribed membranes. Diagnosis: Human parainfluenza virus three pneumonia with associated alveolar proteinosis. Case 3 - Figure 1 - Chest radiograph taken two day prior to death demonstrates bilateral patchy opacities consistent with consolidation. Case 3 - Figure 2 - The lungs at the postmortem examination showed multiple foci of consolidation. Case 3 - Figure 3 - The alveoli were distended by eosinophilic granular material that was Periodic-acid Schiff positive. Case 3 - Figure 4 - The alveoli were distended by eosinophilic granular material that was Periodic-acid Schiff positive. Case 3 - Figure 5 - Giant cells with complex configurations and multiple nuclei were also seen. Case 3 - Figure 6 - Giant cells with complex configurations and multiple nuclei were also seen. Differential Diagnosis Primary and secondary alveolar proteinosis Pneumocystis carinii pneumonia Giant cell pneumonia, other viral etiologies e.g. respiratory synctial virus, measles virus Cytomegalovirus References Delage G, Brochu P, Pelletier M et al. Giant cell pneumonia caused by parainfluenza virus. J Pediatr 1979; 94: 426-429 Little BW, Tihen WS, Dickerman JD, Craighead JE. Giant cell pneumonia associated with parainfluenza virus type 3 infection. Hum Pathol 1981; 12: 478-481 Nuckols JD. Autopsy findings in umbilical cord blood transplant recipients.Am J Clin Pathol1999 335-342 Tinoveanu G, Dobresco G, Gheorghiu C, Alveolar proteinosis in infants coexisting with viral ( cytomegalovirus and measles virus) and microbial infections. Rev Med Chir Soc Med Nat Iasi. 1972; 76: 187-193 Cortez KJ, Erdmann DD, Peret TC et al. Outbreak of human parainfluenza virus 3 infections in a hematopoietic stem cell transplant population. J Infect Dis. 2001; 184: 1093-1097 Cordonnier C, Fleury-FeithJ, Escudier E, Atassi K, Bernaudin JF. Secondary alveolar proteinosis is a reversible cause of respiratory failure in leukemic patients. Am J Respir Crit Care Med 1994; 149: 788-794 Singh G, Katyal SL, Bedrossian CW, Rogers RM. Pulmonary alveolar proteinosis: staining for surfactant apoprotein in alveolar proteinosis and in conditions simulating it.