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The Hepatopulmonary Syndrome Masquerading as Pulmonary Langerhans-Cell Histiocytosis

Stephen H. Caldwell, MD; Kenneth Brantley, MD; John Dent, MD; Robert C. Keeley, MD; Timothy Pruett, MD; John F. Angle, MD; Michael Gaffey, MD; Peter Waldron, MD, Hepatopulmonary Syndrome Study Group.
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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. Requests for Reprints: 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.

Copyright ©2004 by the American College of Physicians

Ann Intern Med. 1994;121(1):34-36. doi:10.7326/0003-4819-121-1-199407010-00006
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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, 34]. 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 [67] and has a poor prognosis [8]. Liver transplantation may reverse the condition in 3 to 12 months [911]. To our knowledge, the hepatopulmonary syndrome has not been reported in patients with Langerhans-cell histiocytosis.

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Figure 1.

Liver biopsy specimen showing heavy portal fibrosis and a regenerating nodule (original magnification, × 50). Chest radiograph showing a mild increase in the lower lung field markings, particularly in the right lower lung field. . Pulmonary angiogram showing the vasculature well visualized without evidence of pulmonary hypertension. The angiogram suggests a “spongy” texture to the vasculature.

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