S2660 A Case of Hepatopulmonary Syndrome Requiring Living Donor Liver Transplantation in a Patient With Nodular Regenerative Hyperplasia and Autoimmune Lymphoproliferative Syndrome-Associated Granulomatous Hepatitis Without Cirrhosis

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Introduction: Nodular regenerative hyperplasia (NRH) is a non-cirrhotic cause of portal hypertension that can rarely lead to hepatopulmonary syndrome (HPS). We report on a patient with a rare underlying Autoimmune Lymphoproliferative Syndrome (ALPS), granulomatous hepatitis, and NRH who developed HPS requiring living donor liver transplantation (LDLT). Case Description/Methods: A 34-year-old male with history of ALPS and prior diagnosis of non-necrotizing granulomatous hepatitis with perisinusoidal fibrosis, presented to our clinic for evaluation of worsened liver enzyme elevations. He was asymptomatic with labs notable for ALT 145 U/L, AST 228 U/L, ALP 308 U/L, and total bilirubin 3.0 mg/dL. His liver enzymes did not improve with a trial of high dose steroids, and repeat liver biopsy was suggestive of nodular regenerative hyperplasia. He later developed signs of portal hypertension on imaging as well as esophageal varices on upper endoscopy, with recurrent bleed requiring Transjugular Intrahepatic Portosystemic Shunting (TIPSS) placement. He then developed chronic hypoxia. Echocardiogram with agitated saline study revealed right to left intrapulmonary shunting, consistent with a diagnosis of HPS. The patient’s symptoms quickly progressed with worsening hypoxia requiring continuous oxygen therapy, PaO2 was 48 on room air. He was listed for liver transplantation with exception points for HPS (native MELD-Na = 13, listed at 27). 183 days after listing, he underwent LDLT with complete resolution of hypoxia within 25 days post-transplant. The explant confirmed the absence of cirrhosis and the presence of granulomatous hepatitis and NRH (Figure 1). Discussion: HPS is most commonly associated with cirrhosis. This case highlights a rare instance of HPS in a patient without cirrhosis, but with NRH and concomitant granulomatous hepatitis. LDLT led to a rapid reversal of his HPS.Figure 1.: Histological section of the explanted native liver revealing subtle nodules without fibrous septa composed of hepatocytes arranged in plates 1-2 cells thick (*). Hepatocytes between nodules are compressed into thin, atrophic plates (arrows). (H&E, original magnification x200).