56-year-old male with pruritus. High gamma GT (240IU/l),
markedly elevated ALP (480IU/l, normal 65-120) and transaminases
(AST: 120IU/l, ALT 140 IU/l). Recent onset icterus with raised billirubin.
(80IU/l, Normal: 3.4-18.8 total billirubin)
Chronic destructive, non-pyogenic cholangitis (so-called primary
billiary cirrhosis) only advances to cirrhosis in final stages.
Histologically there are 4 different stages:
- Stage I: destruction of septal, interlobular and small bile canaliculi
which lie in dense inflammatory infiltrates consisting of plasma
cells (in the vicinity of degenerating bile canaliculi) and lymphocytes
(which partly form follicles). The bile canaliculi can ulcerate
which results in the destruction of the basal lamina. In the given
specimen Stage I is only visible in few portal fields.
- Stage II: proliferation of the ductules, which can degenerate
- Stage III: Vanishing bile duct in centroacinaras well as peripheral
areas, ultimately leading to icterus (clearly visible in the given
The vanishing bile duct leaves scars as well as isolated arterioles
(bile canaliculi are solely supplied with arterial blood)
- Stage IV: cirrhosis. In the given specimen there is only partial
In this specimen all 4 stages are present to a varying extent in
the different portal fields.
In the periphery of the specimen Mallory bodies can be seen near
the degenerated bile canaliculi. Mallory bodies are -when present-
in the periphery of the lobe (In contrast to centroacinar Mallory
bodies in alcoholic hepatitis)
Cholestasis is generally a late symptom. Cholestasis is often more
extensive in ducts and canaliculi of the lobe periphery. In this
given case, centroacinar cholestasis can also be seen, which points
towards a recent onset cholestasis.
Epithelioid cell granulomas, either portal or in the parenchyma
are good prognostic factors.