S5856 When the Clues Don't Add Up: A Case Noncirrhotic Portal Hypertension With Nonspecific Histology Presenting as Variceal Bleeding

Introduction: Clinically significant portal hypertension is defined by HVPG ≥10 mmHg or clinical decompensation. While cirrhosis is the most common cause, any increase in portal resistance can lead to it. Non-cirrhotic portal hypertension (NCPH) is rare, involving portal hypertension without cirrhosis, and requires biopsy, expert pathology, and clinical correlation for diagnosis. Causes include idiopathic factors, infections, immune disorders, medications, or sarcoidosis. We present a complex NCPH case with multiple potential causes. Case Description/Methods: A 49-year-old woman with mesenteric follicular lymphoma (treated with bendamustine/rituximab), common variable immunodeficiency on IVIG, pulmonary sarcoidosis, and chronic thrombocytopenia presented with massive hematemesis and hemorrhagic shock. Symptoms began with crampy epigastric pain followed by approximately 10 cups of hematemesis. She had no prior GI bleeding. History included recent shingles treated with prolonged nonsteroidal anti-inflammatory drug use and untreated reflux. On arrival, HR was 160 bpm, BP 123/90 mmHg, Hgb 10.2 g/dL, lactate 3.7 mmol/L, and platelets 145K. Computed tomography abdomen showed splenomegaly, portal vein dilation, esophageal and gastric varices, and pancreatitis. She received fluids, blood products, intravenous octreotide and pantoprazole, and was admitted to the intensive care unit. Esophagogastroduodenoscopy (EGD) revealed large esophageal varices (banded), portal hypertensive gastropathy, Forrest class III gastric ulcers, and a Mallory-Weiss tear. Initial percutaneous liver biopsy showed stage 0–1 fibrosis with no evidence of cirrhosis, infiltration, steatosis, metal deposition, or sarcoid involvement. FibroScan showed F4 fibrosis. Hepatitis panel was negative. IR performed a hepatic venogram showing HVPG was 25 mmHg and patent hepatic and portal veins. Transjugular biopsy revealed grade 1–2/4 focal periportal activity with focal septal stage 2–3/4 fibrosis, no steatosis, granulomas, Schistosoma eggs, or evidence of autoimmune hepatitis. She required multiple follow-up EGDs and repeat band ligations. Discussion: This case highlights the diagnostic challenge of NCPH in a patient with multiple risk factors. It emphasizes the importance of liver biopsy, expert pathology, and clinical correlation in distinguishing NCPH from cirrhosis. Despite advanced fibrosis on FibroScan, biopsy showed no cirrhosis, underscoring the role of histology. Multiple band ligations reflect ongoing portal hypertension management complexity. This case provides an educational example of comprehensive evaluation in atypical portal hypertension.