Histology Course S II 8 Department of Pathology, University of Zurich Histokurs

S II 8 primary biliary cirrhosis HE further information (German) on HiPaKu
  ICD-10: K74.3

 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 again.

- Stage III: Vanishing bile duct in centroacinaras well as peripheral areas, ultimately leading to icterus (clearly visible in the given specimen).
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 cirrhotic rearrangement.

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.