Nodular Regenerative Hyperplasia: A Case of Rare Prognosis

Abstract

Introduction: Nodular regenerative hyperplasia (NRH) is a known etiology of noncirrhotic portal hypertension. Cases of biopsy-proven NRH in human immunodeficiency virus (HIV)-positive patients have been described. While these patients often have normal synthetic liver function, several reports described disease progression to liver failure. Case: We here present a 26-year-old woman with history of congenital HIV on antiretroviral therapy complicated by Pneumocystis carinii pneumonia at age 14. CD4 counts have been >300 with undetectable viral load. She was referred to our Hepatology service for evaluation of splenomegaly, elevated liver tests, and thrombocytopenia. On initial presentation, she reported easy bruising and gingival bleeding, and abdominal imaging showed evidence of portal hypertension without associated cirrhosis. Upper endoscopy was significant for large esophageal varices without bleeding stigmata. Liver biopsy showed minimal fibrosis around the portal areas without significant inflammation. The lobules showed focal zones of thin hepatocyte plates on reticulin stain with adjacent areas showing mild regenerative changes. The diagnosis of NRH was made and patient was placed on propranolol for variceal bleeding prophylaxis. Two years later, the patient presented with bleeding gastric varices warranting transjugular intrahepatic portosystemic shunt. Postprocedure course was complicated by mild encephalopathy. Subsequent magnetic resonance imaging showed a 1.7 × 1.3 cm lesion suggestive of hepatocellular carcinoma (HCC). The patient was deemed to be a candidate for liver transplantation, and she is now delisted due to ongoing pregnancy. Conclusion: This report describes the first case of HCC in an HIV patient with NRH. The possible association of NRH with HCC warrants further investigation.

Keywords: Hepatocellular carcinoma; Hepatoma; Nodular regenerative hyperplasia; Noncirrhotic portal hypertension.

Figure 1.

Hematoxylin and eosin stain of liver biopsy. The hepatocytes between the nodules are compressed and atrophic (blue arrow). The liver biopsy slide does not belong to the case reported in this article as its slides were accidentally damaged under unexpected circumstances. The slide is provided to clearly illustrate the histologic features of nodular regenerative hyperplasia.

Figure 2.

Reticulin stain of liver biopsy. The reticulin network is compressed in the parenchyma between the nodules (red arrows). Hepatocytes within nodules are arranged in plates that are 1 to 2 cells thick (blue arrow). The liver biopsy slide does not belong to the case reported in this article as its slides were accidentally damaged under unexpected circumstances. The slide is provided to clearly illustrate the histologic features of nodular regenerative hyperplasia.

Figure 3.

Hepatocellular carcinoma (arrows) with arterial enhancement (arterial phase).

Figure 4.

Washout during the venous phase.

Figure 5.

Hepatocellular carcinoma (arrows) on hepatobiliary phase.