Contributed by Randall G. Lee, M.D.
PATIENT HISTORY: Per referral letter, the patient is a 69-year-old female who has been generally well but has had an elevated alkaline phosphatase and transaminases and suffers from fatigue. A work-up has disclosed negative or non-reactive serologic markers for viral hepatitis. She has a significantly positive AMA antibody (1:640) and she has a low titer positive ANA. Review of outside material.

Final Diagnosis (Case 38)

LIVER, NEEDLE BIOPSY -
  1. HISTOLOGIC FEATURES CHARACTERISTIC OF PRIMARY BILIARY CIRRHOSIS.
  2. BRIDGING FIBROSIS WITH FOCAL ARCHITECTURAL DISTORTION (STAGE III).

Previous Biopsies on this Patient:
None

TPIS Related Resources:
Knodell Scoring
Liver Transplant Topics


Gross Description - Case 38

The specimen consists of four (4) consult slides and one (1) paraffin block. No surgical pathology report is received with the specimen.


Microscopic Description - Case 38

The liver biopsy demonstrates hepatic architecture distorted by irregular portal fibrosis with focal bridging fibrosis and early nodular formation. Cirrhosis has not developed, however. The portal tracts predominantly demonstrate dense ductular proliferation with a mixed inflammatory response including neutrophils and lymphocytes. Mild lymphocytic type piecemeal necrosis is identified. In occasional portal tracts, no definite intralobular bile duct is noted, whereas other tracts shows ducts with epithelial eosinophilia and focal lymphocytic infiltration. In addition, a medium-sized duct demonstrates a so-called florid duct lesion with duct rupture, adjacent inflammation including epithelioid histiocytes and eosinophils, and intraepithelial lymphocyte inflammation. These later duct changes are most helpful in establishing a diagnosis of primary biliary cirrhosis, and are consistent with the clinical setting and AMA positivity.

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