S5647 Not All Roads Lead to Cirrhosis: A Portal Hypertension Puzzle

Introduction: Portal hypertension (PTN) is marked by the elevation of flow or resistance within the hepatic portal system. PTN is clinically significant when the pressure gradient is greater than 10 mmHg. The most common cause of PTN is cirrhosis due to alcohol or viral hepatitis.1 However, hematologic disorders, including myeloproliferative syndromes, can result in non-cirrhotic portal hypertension. We introduce a patient who presented with symptoms of hematemesis and hematochezia. We hope to emphasize the importance of considering hematological/oncological differentials as a cause of PTN, particularly in elderly patients with atypical presentations. Case Description/Methods: Patient is an 82-year-old man with a history notable for chronic myelomonocytic leukemia (CMML), and esophageal varices. He initially presented to an outside hospital with hematemesis and hematochezia. An episode of GI bleeding occurred one month prior, for which he underwent endoscopic coiling. Patient was transferred to our facility for consideration of possible transjugular intrahepatic portosystemic shunt (TIPS) procedure. On admission, initial labs were notable for white blood cell count 29, and Hemoglobin 4.7. His physical exam showed abdominal distension with positive fluid wave. Initial management included transfusion of PRBCs, intravenous (IV) octreotide, and IV pantoprazole. Patient underwent esophagogastroduodenoscopy which revealed non-bleeding esophageal varices. He was evaluated for TIPS, however, the procedure was deferred after HVPG was found to be 6 mmHg. Patient instead underwent plug-assisted retrograde transvenous obliteration (PARTO) for treatment of his esophageal varices. Given his significant leukocytosis and hepatic venous pressure gradient (HVPG) findings, hematology/oncology was consulted. They had a high suspicion of possible myeloproliferative neoplasm (MPN) as the underlying etiology for both the leukocytosis and portal hypertension. A bone marrow biopsy showed JAK2 positive primary myelofibrosis. The patient was discharged with follow-up planned with hepatology and oncology for further treatment planning. Discussion: This case demonstrates a rare presentation of MPN-associated PTN. Our patient presented with findings that initially pointed toward decompensated cirrhosis. However, his findings prompted further workup, revealing MPN-associated PTN. This case also highlights the importance of avoiding anchoring patients when evaluating PTN. Reference 1. Bloom S, Kemp W, Lubel J. Portal hypertension: Pathophysiology, diagnosis and management. Intern Med J. 2015 Jan;45(1):16-26.