Case 616 -- A 23 year old male with portal hypertension in the stomach and an erythematous proximal jejunum

Contributed by Gerard J Oakley III MD and Alyssa Krasinskas, MD


The patient is a 23 year old male with a history of Crohn's disease and primary sclerosing cholangitis since age 15. In 2003 he received a living donor liver, which had to be replaced with an orthotopic liver 10 days after transplant due to complications. Over the previous month, his liver enzymes had been trending upwards, worrisome for return of primary liver disease, versus possible stricture at the biliary-enteric anastomosis. Aside from mild generalized jaundice, no discrete worrisome skin lesions were noted. He complained of abdominal and back pain, the latter believed to be a mild inflammation of spinal joints. An upper endoscopy was performed, and significant findings included a patent anastomosis, evidence of portal hypertension in the stomach, and an erythematous proximal jejunum, which was biopsied. Of note, his pre-operative labs showed a microcytic anemia (hemoglobin 10.5 g/dL and MCV 73.9 fL) with an expanded red cell width of 19.5%, which was being treated with oral iron supplementation.


The specimen was received in one part, and consisted of a single 0.3 cm fragment of soft tan tissue, which was entirely submitted.


The specimen showed small intestinal mucosa with preserved villous architecture and no evidence of acute or chronic inflammation (Figure 1). No parasites were seen, and there was no evidence of acute mucosal hemorrhage. However, the tips of the showed an accumulation of "chunky" golden-brown to black pigment in engorged, bland appearing macrophages in the lamina propria of these villi (Figure 2). Iron staining was positive in this pigment (Figure 3), but not in epithelial cells or around vessels.


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