P1083 - A Rare Case of Non-Cirrhotic Portal Hypertension Caused by Necrobiotic Xanthogranuloma

Phillip Leff, DO¹, Rida Nadeem, MD, MSPH², Brett A. Dinner, MD³, Ria Kundu, MD⁴, Naim Alkhouri, MD², Anita Kohli, MD^2
1Creighton University, Scottsdale, AZ; ²Arizona Liver Health, Chandler, AZ; ³Creighton University, Phoenix, AZ; ⁴Banner Health, Mesa, AZ

Introduction: Portal hypertension is commonly a sign of cirrhotic liver disease, and less commonly seen with obstructions i.e. clots. However, a small portion of these patients can have significant portal hypertension without cirrhosis, or veinous obstruction. This is a case of a rarely identified cause of non-cirrhotic portal hypertension (NCPH) in a patient with necrobiotic xanthogranuloma (NXG).

Case Description/Methods: Patient was a 70-year-old male presented to an outpatient hepatology clinic for liver fibrosis of unknown origin seen on imaging. Patient roughly 30 years prior had developed migratory polyarticular pain, and was found to have an elevated IgG level. A bone marrow biopsy showed 40% cellularity, normal trilineage hematopoiesis, megakaryocytes 2.4/hpf with 2.6 polytypic plasma cells. Myeloma FISH was negative. Patient presented to dermatology for round erythematous pruritic skin lesions, a biopsy was performed demonstrating NXG. Patient was found to have significant fibrosis and splenomegaly 16.9cm on ultrasound. Liver disease workup for viral hepatitis, autoimmune hepatitis, A1ATD, Wilsons, hemochromatosis, and storage disorders were all negative.



Patient underwent a liver biopsy which showed stage 2-3 liver fibrosis and hepatic plate architectural abnormality with sinusoidal dilation, congestion and lobular vascular shunt. An ultrasound doppler of liver was performed showing coarsened echotexture of the liver consistent with a history of cirrhosis. The portal vein is dilated, with hepatopedal flow in the portal vein. An MRI w/wo contrast showed small biliary hemartoma in inferior right liver, mild fibrotic change seen in the peripheral right liver, and mild periportal edema. no intrahepatic or extrahepatic ductal dilatation, and spleen at 15.5 cm. Given the patient’s negative workup on presentation to our hepatology clinic the elevation in ALT/AST were presumed to be secondary to NXG. Patient was started on a trial of 12 weeks of budesonide. There was no significant decrease in ALT/AST on the treatment with budesonide. Repeat bloodwork status post budesonide showed ALT/AST 26/71.

Discussion: The most common causes of NCPH is chronic infections, medications, thrombophilia, autoimmune disorders, and genetic disorders. NXG should remain on the differential for patients with NCPH without a known cause, as half cases of NCPH do not have a formal diagnosis to account for the patient’s portal hypertension.


Disclosures:


Phillip Leff, DO¹, Rida Nadeem, MD, MSPH², Brett A. Dinner, MD³, Ria Kundu, MD⁴, Naim Alkhouri, MD², Anita Kohli, MD². P1083 - A Rare Case of Non-Cirrhotic Portal Hypertension Caused by Necrobiotic Xanthogranuloma, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.