The Hepatopulmonary Syndrome Masquerading as Pulmonary Langerhans-Cell Histiocytosis
Authors: Stephen H. Caldwell
Journal: Annals of Internal Medicine (1994)

Brief Communications1 July 1994The Hepatopulmonary Syndrome Masquerading as Pulmonary Langerhans-Cell HistiocytosisStephen H. Caldwell, MD, Kenneth Brantley, MD, John Dent, MD, Robert C. Keeley, MD, Timothy Pruett, MD, John F. Angle, MD, Michael Gaffey, MD, and Peter Waldron, MD, and Hepatopulmonary Syndrome Study Group.Stephen H. Caldwell, MDFrom the University of Virginia Health Sciences Center, Charlottesville, Virginia, and Community Hospital of the Roanoke Valley, Roanoke, Virginia.For members of the Hepatopulmonary Syndrome Study Group and current author addresses, see end of text.Search for more papers by this author, Kenneth Brantley, MDFrom the University of Virginia Health Sciences Center, Charlottesville, Virginia, and Community Hospital of the Roanoke Valley, Roanoke, Virginia.For members of the Hepatopulmonary Syndrome Study Group and current author addresses, see end of text.Search for more papers by this author, John Dent, MDFrom the University of Virginia Health Sciences Center, Charlottesville, Virginia, and Community Hospital of the Roanoke Valley, Roanoke, Virginia.For members of the Hepatopulmonary Syndrome Study Group and current author addresses, see end of text.Search for more papers by this author, Robert C. Keeley, MDFrom the University of Virginia Health Sciences Center, Charlottesville, Virginia, and Community Hospital of the Roanoke Valley, Roanoke, Virginia.For members of the Hepatopulmonary Syndrome Study Group and current author addresses, see end of text.Search for more papers by this author, Timothy Pruett, MDFrom the University of Virginia Health Sciences Center, Charlottesville, Virginia, and Community Hospital of the Roanoke Valley, Roanoke, Virginia.For members of the Hepatopulmonary Syndrome Study Group and current author addresses, see end of text.Search for more papers by this author, John F. Angle, MDFrom the University of Virginia Health Sciences Center, Charlottesville, Virginia, and Community Hospital of the Roanoke Valley, Roanoke, Virginia.For members of the Hepatopulmonary Syndrome Study Group and current author addresses, see end of text.Search for more papers by this author, Michael Gaffey, MDFrom the University of Virginia Health Sciences Center, Charlottesville, Virginia, and Community Hospital of the Roanoke Valley, Roanoke, Virginia.For members of the Hepatopulmonary Syndrome Study Group and current author addresses, see end of text.Search for more papers by this author, and Peter Waldron, MDFrom the University of Virginia Health Sciences Center, Charlottesville, Virginia, and Community Hospital of the Roanoke Valley, Roanoke, Virginia.For members of the Hepatopulmonary Syndrome Study Group and current author addresses, see end of text.Search for more papers by this author, and Hepatopulmonary Syndrome Study Group.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-121-1-199407010-00006 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail Langerhans-cell histiocytosis (which can take the form of histiocytosis X, eosinophilic granuloma, Abt-Letterer-Siwe disease, and Hand-Schuller-Christian disease) may involve the bone, lung, liver, hypothalamus, and pituitary [1]. Pulmonary failure is the leading cause of death in long-term survivors [2]. Liver involvement, seen in 65% of patients with the disease, may progress to sclerosing cholangitis and cirrhosis [1, 3, 4]. Liver biopsy specimens may show cirrhosis without active histiocyte infiltration [4]. Liver transplantation in hepatobiliary Langerhans cell histiocytosis has been reported [5]. The hepatopulmonary syndrome is characterized by intrapulmonary vasodilatation and hypoxemia in patients with portal hypertension [6, 7] and has ...References1. Berry DH, Becton DL. Natural history of histiocytosis-8. Hematol Oncol Clin North Am. 1987; 1:23-34. Google Scholar2. Komp DM. Long-term sequelae of histiocytosis-8. Am J Ped Hematol Oncol. 1981; 3:165-8. Google Scholar3. Greenberger JS, Crocker AC, Vawter G, Jaffe N, Cassady JR. Results of treatment of 127 patients with systemic histiocytosis (Letterer-Siwe syndrome, Schuller-Christian syndrome and multifocal eosinophilic granuloma). 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Eriksson LS, Soderman C, Ericzon BG, Eleborg L, Wahren J, Hedenstierna G, et al. Normalization of ventilation/perfusion relationships after liver transplantation in patients with decompensated cirrhosis: evidence for a hepatopulmonary syndrome. Hepatology. 1990; 12:1350-7. Google Scholar10. Stoller JK, Moodie D, Schiavone WA, Vogt D, Broughan T, Winkelman E, et al. Reduction of intrapulmonary shunt and resolution of digital clubbing associated with primary biliary cirrhosis after liver transplantation. Hepatology. 1990; 11:54-8. Google Scholar11. Eriksson LS. Is intrapulmonary arteriovenous shunting and hypoxemia a contraindication for liver transplantation? Hepatology. 1991; 14:575-6. Google Scholar12. Ha SY, Helms P, Fletcher M, Broadbent V, Pritchard J. Lung involvement in Langerhans' cell histiocytosis: prevalence, clinical features, and outcome. Pediatrics. 1992; 89:466-9. Google Scholar13. Basset F, Corrin B, Spencer H, Lacronique J, Roth C, Soler P, et al. Pulmonary histiocytosis X. Am Rev Respir Dis. 1978; 118:811-20. Google Scholar14. Friedman PJ, Liebow AA, Sokoloff J. Eosinophilac granuloma of lung. Clinical aspects of primary histiocytosis in the adult. Medicine (Baltimore). 1981; 60:385-96. Google Scholar15. Marcy TW, Reynolds HY. Pulmonary histiocytosis X. Lung. 1985; 163:129-50. Google Scholar16. Krowka MJ, Cortese DA. Pulmonary aspects of chronic liver disease and liver transplantation. Mayo Clin Proc. 1985; 60:407-18. Google Scholar17. Hind CR, Wong CM. Detection of pulmonary arteriovenous fistulae in patients with cirrhosis by contrast 2D echocardiography. Gut. 1981; 22:1042-5. Google Scholar Author, Article, and Disclosure InformationAffiliations: From the University of Virginia Health Sciences Center, Charlottesville, Virginia, and Community Hospital of the Roanoke Valley, Roanoke, Virginia.For members of the Hepatopulmonary Syndrome Study Group and current author addresses, see end of text.Corresponding Author: Stephen H. Caldwell, MD, Division of Gastroenterology, Hepatology, and Nutrition, Box 145, University of Virginia Health Sciences Center, Charlottesville, VA 22908.Acknowledgments: The authors thank Ms. Chris Spillman for assistance in manuscript preparation and Dr. David Stone for his review of this manuscript. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byOutcomes of liver transplantation in patients with hepatopulmonary syndrome in the pre and post-MELD eras: A systematic reviewHepatopulmonary Disorders: Gas Exchange and Vascular Manifestations in Chronic Liver DiseaseSyndrome hépatopulmonaire et pneumopathie interstitielle diffuse idiopathiqueLangerhans cell histiocytosisTHE HEPATOPULMONARY SYNDROMEHepatopulmonary Syndrome With Progressive Hypoxemia as an Indication for Liver Transplantation: Case Reports and Literature ReviewTHE HEPATOPULMONARY SYNDROMEHepatopulmonary syndrome: What are we learning from interventional radiology, liver transplantation, and other disorders? 1 July 1994Volume 121, Issue 1Page: 34-36KeywordsBiopsyCirrhosisLiverLiver diseasesLungsPulmonary arteriesPulmonary diseasesThoraxThyroxineVasodilation Issue Published: 1 July 1994 Copyright & PermissionsCopyright © 1994 by American College of Physicians. 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