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Hepatopulmonary Syndrome Associated With Large Granular Lymphocytic Leukemia FREE TO VIEW

Tamara Dahhan, DO; Armand Ryden, MD; Nader Kamangar, MD
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Olive View UCLA Medical Center, Sylmar, CA

Chest. 2013;144(4_MeetingAbstracts):969A. doi:10.1378/chest.1701618
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SESSION TITLE: Miscellaneous Student/Resident Case Report Posters II

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Hepatopulmonary Syndrome (HPS) is a disorder comprised of liver disease, pulmonary vasculature dilation, and hypoxemia. We present a unique case of HPS caused by Large Granular Lymphocytic Leukemia (LGL), a rare lymphoproliferative syndrome, causing infiltrating liver disease without cirrhosis.

CASE PRESENTATION: A 32-year-old male with longstanding history of pancytopenia, hypersplenism, enlarged gastric and esophageal varices, presented with progressive exertional dyspnea and hypoxemia over one and a half years. He had a marked decrease in exercise tolerance and progressive hypoxemia requiring 4 liters via nasal cannula. A previous bone marrow biopsy was suspicious for LGL. Family and social histories were unremarkable. Physical exam was notable for an oxygen saturation of 87-93% on room air with associated mild orthodeoxia; spider angiomata on chest; severe digital clubbing; and palpable splenomegaly. Laboratory was significant for mild elevation in serum bilirubin and INR; thrombocytopenia and a markedly elevated A-a gradient. Thoracic CT was remarkable for ectatic distal pulmonary vasculature, most notable in in the lower lung fields. Pulmonary function testing was notable for severely reduced DLCO. Transthoracic echocardiogram with bubble was significant for evidence of right to left shunt. Right heart catheterization showed normal hemodynamics and a shunt fraction of 1.03, suggesting an absence of intracardiac shunt. Despite this, given high clinical suspicion for intrapulmonary shunt, a microaggregated albumin nuclear scan was obtained which showed a right to left shunt of 34%. The patient underwent a liver biopsy which showed focal nodules surrounded by fibrous tissue, with some inflammatory cells, likely representing LGL in the liver.

DISCUSSION: HPS is a well known complication of cirrhotic liver disease that presents with hypoxemia. HPS has also been described in both acute and non-cirrhotic liver disease. The diagnosis of HPS in this case was more difficult due to the absence of obvious liver disease on imaging and laboratory testing. Furthermore, the use of the cardiac catheterization to characterize the shunt was likely misleading. Our review of the literature found no reported cases of HPS in LGL, while LGL itself has been rarely reported to cause infiltrative liver disease.

CONCLUSIONS: HPS can complicate any liver disease, however the diagnosis can be elusive when the manifestations of the liver are subtle, particularly when the liver disease is from a rare etiology.

Reference #1: Rodriguez-Roisin R, and Krowka MJ. Hepatopulmonary Syndrome - A Liver-Induced Lung Vascular Disorder. NEJM 2008;358:2378-87

DISCLOSURE: The following authors have nothing to disclose: Tamara Dahhan, Armand Ryden, Nader Kamangar

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