During laparoscopy performed for other reasons, a surgeon is staled to see a uniformly black liver. Which of the following is the most likely diagnosis?
Correct Answer: Dubin-Johnson syndrome
Description: Dubin-Johnson syndrome is a relatively mild, hereditary conjugated hyperbilirubinemia that has the distinctive feature of causing the liver to turn black. The same black pigment can be seen in microscopic sections. The origin of the black pigment is not fully understood, but the genetic defect responsible for Dubin-Johnson syndrome has recently been identified, and appears to involve a defect in the canalicular organic anion transpoer. Patients homozygous for Dubin-Johnson syndrome may also have significant amounts of unconjugated bilirubin in the serum, presumably due to deconjugation of conjugated bilirubin in the hepatobiliary system. Angiosarcoma can produce a dark red liver due to increased blood and hemorrhage. Hemochromatosis can produce a dark red to brown liver. Malignant melanoma can produce black nodules in the liver, but would not be expected to produce a uniformly black liver. Ref: Wyatt C., Kemp W.L., Moos P.J., Burns D.K., Brown T.G. (2008). Chapter 15. Pathology of the Liver, Gallbladder, and Pancreas. In C. Wyatt, W.L. Kemp, P.J. Moos, D.K. Burns, T.G. Brown (Eds), Pathology: The Big Picture.
Category:
Pathology
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