Virchows Archiv A

, Volume 376, Issue 2, pp 165–174 | Cite as

Degenerative processes in the pathogenesis of pulmonary alveolar lipoproteinosis

  • Danielle Jacobovitz-Derks
  • Bryan Corrin


Electron microscopy in an infant of 4 months with pulmonary alveolar lipoproteinosis showed filling of the alveoli with osmiophilic lamellar bodies. Similar structures were present in the cytoplasm of type I alveolar epithelial cells and to a lesser extent in the capillary endothelium and interstitium. These changes represent widespread degenerative processes in the lung caused by an unidentified cytotoxic agent. In this patient the disease is comparable to the drug-induced cytotoxic animal model and differs from the dust-induced hypersecretory animal model.

Key words

Lung Electron microscopy Pulmonary alveolar proteinosis Degeneration 


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  1. Basset, F., Soler, P., Turiaf, J.: Etude ultrastructurale du contenu alvéolaire dans la protéinose alvéolaire pulmonaire et dans les pseudoprotéinoses. Ann. Med. interne. 124, 279–290 (1973)Google Scholar
  2. Buechner, H.A., Ansari, A.: Acute silico-proteinosis. A new pathologic variant of acute siliconsis in sandblasters, characterised by histologic features resembling alveolar proteinosis. Dis. Chest. 55, 274–284 (1969)Google Scholar
  3. Carnovale, R., Zomoza, J., Goldman, A.M., Luna, M.: Pulmonary alveolar proteinosis: its association with haematologic malignancy and lymphoma. Radiology 122, 303–306 (1977)Google Scholar
  4. Corrin, B.: Aluminium pneumoconiosis. MD Thesis, University of London (1962)Google Scholar
  5. Corrin, B., King, E.: Inhalation experiments comparing the action of quartz and cristobalite on the rat lung. Proc 113th meeting Pathological Society of Great Britain and Ireland, p. 14 (issued with J. Path. Bact. 92) (1966)Google Scholar
  6. Corrin, B., King, E.: Experimental endogenous lipid pneumonia and silicosis. J. Path. 97, 325–330 (1969)Google Scholar
  7. Corrin, B., King, E.: Pathogenesis of experimental pulmonary alveolar proteinosis. Thorax 25, 230–236 (1970)Google Scholar
  8. Costello, J.F., Moriarty, D.C., Branthwaite, M.A., Turner-Warwick, M., Corrin, B.: Diagnosis and management of alveolar proteinosis: the role of electron microscopy. Thorax 30, 121–131 (1975)Google Scholar
  9. Davidson, J.M., McCleod, W.M.: Pulmonary alveolar proteinosis. Brit. J. Dis. Chest. 63, 13–28 (1969)Google Scholar
  10. Gil, J., Weibel, E.R.: Improvements in demonstration of lining layer of lung alveoli by electron microscopy. Resp. Physiol. 8, 13–36 (1969)Google Scholar
  11. Gray, E.S.: Autoimmunity in childhood pulmonary alveolar proteinosis. Brit. Med. J. 4, 296–297 (1973)Google Scholar
  12. Gross, P., deTreville, R.T.P.: Alveolar proteinosis: its experimental production in rodents. Arch. Path. 86, 255–261 (1968)Google Scholar
  13. Hawkins, J.E., Savard, E.V., Ramirez-Rivera, J.: Pulmonary alveolar proteinosis: origin of protein in pulmonary washings. Amer. J. Clin. Path. 48, 14–17 (1967)Google Scholar
  14. Heath, D., Smith, P., Hasleton, P.S.: Effects of chlorphentermine on the rat lung. Thorax 28, 551–558 (1973)Google Scholar
  15. Heppleston, A.G.: Atypical reaction to inhaled silica. Nature 213, 199 (1967)Google Scholar
  16. Heppleston, A.G., Wright, N.A., Stewart, J.A.: Experimental alveolar lipoproteinosis following the inhalation of silica. J. Path. 101, 293–307 (1970)Google Scholar
  17. Heppleston, A.G., Young, A.E.: Alveolar lipoproteinosis: An ultrastructural comparison of the experimental and human forms. J. Path. 107, 107–117 (1971)Google Scholar
  18. Heppleston, A.G., Fletcher, K., Wyatt, I.: Changes in the composition of lung lipids and the “turnover” of dipalmitoyl licithin in experimental alveolar lipoproteinosis induced by inhaled quartz. Brit. J. exp. Path. 55, 384–395 (1974)Google Scholar
  19. Hoffmann, E.O., Lamberty, J., Pizzolata, P., Coover, J.: The ultrastructure of acute silicosis. Arch. Path. 96, 104–107 (1973)Google Scholar
  20. Lakshminarayan, S., Schwarz, M.I., Stanford, R.E.: Unsuspected pulmonary alveolar proteinosis complicating acute myelogenous leukemia. Chest 69, 433–435 (1976)Google Scholar
  21. Lüllmann-Rauch, R., Reil, G.H., Rossen, E., Seiler, K.U.: The ultrastructure of rat lung changes induced by an anoretic drug (Chlorphentermine). Virchows Arch. B Zellpath. 11, 167–181 (1972)Google Scholar
  22. McClenahan, J.B., Mussenden, R.: Pulmonary alveolar proteinosis. Arch. Intern. Med. 133, 284–287 (1974)Google Scholar
  23. Mendenhall, R.M., Sun, C.N.: Lining of lung alveoli as a structure. Nature 185, 713–714 (1964)Google Scholar
  24. Ramirez-R., J., Harlan, W.R.: Pulmonary alveolar proteinosis: nature and origin of alveolar lipid. Am. J. Med. 45, 502–512 (1968)Google Scholar
  25. Rosen, S.H., Castleman, B., Liebow, A.A.: Pulmonary alveolar proteinosis. New Eng. J. Med. 258, 1123–1142 (1958)Google Scholar
  26. Schober, R., Kosek, J.C., Bensch, K.G.: Origin of the membranaeous intra-alveolar material in pulmonary alveolar proteinosis. Lab. Invest. 30, 388–389 (1974)Google Scholar
  27. Smith, P., Heath, D., Hasleton, P.S.: Electron microscopy of chlorphentermine lung. Thorax 28, 559–566 (1973)Google Scholar
  28. Vijeyaratnam, G.S., Corrin, B.: Pulmonary alveolar proteinosis developing from desquamative interstitial pneumonia in long term toxicity studies of iprindole in the rat. Virchows Arch. Abt. A Path. Anat. 358, 1–10 (1973)Google Scholar
  29. Xipell, J.M., Ham, K.N., Price, C.G., Thomas, D.P.: Acute silico-lipoproteinosis. Thorax 32, 104–111 (1977)Google Scholar

Copyright information

© Springer-Verlag 1977

Authors and Affiliations

  • Danielle Jacobovitz-Derks
    • 1
    • 2
  • Bryan Corrin
    • 1
    • 2
  1. 1.Hopital St PierreBrussels
  2. 2.Department of Morbid AnatomySt Thomas' HospitalLondonEngland

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