Anatomic Pathology: Liver Pathology

228) The postmortem section of liver shown in the image was MOST likely from a patient whose terminal course was characterized by:

• Bile ductular cholestasis is characterized by proliferation of periportal bile ductular structures (derived from activated hepatic progenitor cells) that contain inspissated bile concretions. Although bile ductular cholestasis is pathognomonic for sepsis, the pathogenesis of this lesion is uncertain. In the older literature of the twentieth century, the lesion was often clinically termed cholangitis lenta and referred to the presence of jaundice occurring in an individual with bacteremia secondary to valvular endocarditis (i.e., endocarditis lenta).

• One hypothesis regarding the pathogenesis of bile ductular cholestasis in the setting of sepsis is that the rapidity of periportal bile flow emptying into the interlobular bile ducts combined with dehydration (and possibly altered bile salt or microbial flora in the periportal regions) results in concentrated bile concretions that localize within newly formed ductular structures.

• The major differential diagnosis is large bile duct obstruction, which should always be excluded clinically.

Histologic caveat: In a neonate with jaundice, bile ductular cholestasis (particularly where proliferated bile ductular structures are readily evident) is highly suggestive and supportive of the diagnosis of extrahepatic biliary atresia.

Geier A, Fickert P, Trauner M: Mechanisms of disease: mechanisms and clinical implications of cholestasis in sepsis. Nat Clin Pract Gastroenterol Hepatol 2006;3(10):574-585.

Kosters A, Karpen SJ: The role of inflammation in cholestasis: clinical and basic aspects. Semin Liver Dis 2010;30(2):185-194.

Lefkowitch JH: Bile ductular cholestasis: an ominous histopathologic sign related to sepsis and “cholangitis lenta.” Hum Pathol 1982;13(1):19-24.

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