A Rare Cause of Noncirrhotic Intrahepatic Portal Hypertension

Abstract

Question: A 38-year-old woman with history of splenomegaly and thrombocytopenia was referred to the outpatient hepatology office after an extensive negative hematologic and infectious workup. On physical examination, the patient seemed to be comfortable with a blood pressure of 110/65 mm Hg and heart rate of 72 bpm. Her abdominal examination showed an enlarged spleen. A rectal examination revealed brown stool without melena. Laboratory evaluation revealed a white blood cell count of 4,800/mL, hemoglobin of 11.3 g/dL (baseline, 12.2 g/dL), platelets 53 10/mL (baseline, 70 10/mL), creatinine of 0.6 mg/dL, prothrombin time of 12 seconds, and International Normalized Ratio of 1.1. Electrolytes, amylase, lipase, liver chemistry tests, ceruloplasmin, and immunoglobulin levels were normal. Anti-smooth muscle antibody and antimitochondrial antibody were negative. Pertinent review of systems revealed that the patient is a current tobacco user with a 10-year pack history and a former user of intravenous heroine. Abdominal ultrasonography with Doppler revealed a diffusely heterogeneous liver parenchyma and enlarged spleen measuring 20.2 cm with a patent splenic vein (Figure A). Esophagogastroduodenoscopy demonstrated multiple columns of large esophageal varices in the lower third of the esophagus (Figure B). A colonoscopy demonstrated small to medium sized, nonbleeding rectal varices. Both studies were consistent with portal hypertension. A transjugular core liver biopsy was performed and the slides demonstrated liver cell plate atrophy alternating with hepatocellular regeneration (hematoxylin and eosin, and reticulin stains), and focal portal and pericellular fibrosis (trichrome stain). A minimal to mild inflammatory infiltrate composed of lymphocytes and clutters of macrophages containing brown granular refractive material was noted in the portal tracts without evidence of cirrhosis (Figure C, D). No significant bile duct injury, steatosis, granulomas, siderosis, or alpha1-antitrypsin globule was identified on histology. These findings were consistent with nodular regenerative hyperplasia, a condition of noncirrhotic intrahepatic portal hypertension (NCIPH). However, further inquisition of the same region under polarized light revealed multiple scattered, white, birefringent, irregular or needle-shaped material within some of the portal tracts (Figures E [arrow head], F [arrow head]).