Patient History
A 38-year old white male was referred to WVUH with the complaints of itching and scratching all over, jaundice and intermittent dark urine for about a month. About 12 years before the referral, he was clinically diagnosed as having large-bowel Crohn’s disease and started on Azulfidine. Reportedly he was doing well until two months prior, when he had episodes of loose bowel movements 3-4 time per day. Laboratory data were as follows:
| LAB DATA |
Patient |
Normal |
Bilirubin |
7mg/dL |
<1 mg/dL |
Alk Phos |
383 U/L |
30-140 U/L |
AST |
86 U/L |
7-46 U/L |
ALT |
90 U/L |
4-34 U/L |
GGT |
348 U/L |
4-65 U/L |
Ferritin |
181 ng/ml |
15-400 ng/ml |
CEA |
1.8 ng/ml |
0-2.5 ng/ml |
Sed Rate |
18 mm/h |
0-15 mm/h |
His hepatitis profile was negative. ANA and alpha-fetoprotein were negative, gammaglobulin was elevated, PT and PTT were within normal limits. Ultrasound of the abdomen showed a normal bile duct, but a CT scan of the abdomen exhibited dilated intrahepatic bile ducts. Endoscopic retrograde cholangiopancreatography (ERCP) showed that the common bile duct was narrowed proximally near the bifurcation of the right and left main intrahepatic bile ducts. The right intrahepatic bile duct was dilated, whereas the left intrahepatic bile duct was strictured. Percuatneous liver biopsy was performed and the representative features were shown in Figures 1 and 2.
The liver biopsy (figures 1 and 2) showed expanded portal areas with fibrosis which bridged adjacent portal areas, proliferating bile ductules and acute and chronic inflammatory cell infiltration. The trichrome stain in figure 2 highlights the fibrotic bands blue. There was acute cholangitis as well as pericholangitis. Cholestasis was present in and around the portal areas. Thus, a diagnosis of large bile duct obstruction and secondary biliary cirrhosis was made, suspecting a possibility of primary sclerosing cholangitis.
Two months later, he was referred to a liver transplant center, where a CT scan exhibited a possible liver hilar mass and an angiography confirmed the same mass at the right aspect of the hilum and partially obstructing the left intrahepatic bile duct as well. ERCP was performed twice and both times the brushings were negative for malignancy. He was readmitted a month later with increased pruritus and jaundice for possible liver transplant. The next day, brushings from a percutaneous transhepatic cholangiogram and biliary drainage were reported to be positive for malignancy. He received radiation therapy at an accelerated rate. Two more weeks later, he was taken to the operating room for an orthotopic liver transplant. On exploratory laparotomy, he was found to have lymph node metastasis on frozen section, and thus was not deemed an appropriate candidate for transplantation. He expired at home six months later.
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