Evaluation of Langerhans Cell Infiltration by CD1a Immunostain in Liver Biopsy for the Diagnosis of Primary Biliary Cirrhosis.
Rondell P Graham, Thomas C Smyrk, Lizhi Zhang. Mayo Clinic, Rochester, MN
Background: Primary biliary cirrhosis (PBC) is characterized by chronic nonsuppurative destructive cholangitis, and is thought to be a cell-mediated immune reaction. Antigen-presenting cells, including Langerhans cells and dendritic cells, have been found in portal tracts and in bile duct epithelium, and may play a role in the pathogenesis in PBC. The diagnostic value of detecting Langerhans cells in PBC has not been evaluated in large scale studies.
Design: Needle biopsies of the liver from adult patients diagnosed with PBC (n=39), primary sclerosing cholangitis (PSC, n=17), obstructive cholangitis (OC, n=13), chronic viral hepatitis B and C (CVH, n=19) and autoimmune hepatitis (AIH, n=20) at our institution were retrieved. The histologic diagnoses were confirmed by reviewing the H&E sections as well as clinical and laboratory data. An immunohistochemical stain for CD1a was used to detect Langerhans cells. The distribution of CD1a positive Langerhans cell infiltrate was recorded as lobular, portal with bile duct sparing, and epithelial. The numbers of Langerhans cells in each area were counted.
Results: Langerhans cells were rarely seen in the lobular parenchyma in any case. A portal Langerhans cell infiltrate was identified in most cases of PBC (62%). Although it was seen significantly more often in PBC than in AIH (30%) or CVH (10%), there were no significant differences compared to PSC (41%) or OC (62%). Intraepithelial Langerhans cells were identified in 44% of PBC, 18% of PSC, 15% of OC, 15% of AIH, and none of CVH. The numbers of intraepithelial Langerhans cells were significantly higher in PBC than in other conditions (number of intraepithelial Langerhans cells per bile duct in PBC, median= 4, mean=4.9, range=2-11). Using a cut-off of ≥3 Langerhans cells per bile duct as “positive,” gave a sensitivity for PBC of 38% (15/39) and a specificity of 97%, with only rare other biopsies (two AIH) having this density of intraepithelial infiltrate.
Conclusions: A CD1a-positive intraepithelial Langerhans cell infiltrate of ≥3 Langerhans cells in a bile duct is highly specific for PBC, but not very sensitive. This marker may be a useful adjunct to the diagnosis of PBC in cases lacking typical histologic features or serology.
Category: Liver & Pancreas
Monday, February 28, 2011 9:30 AM
Poster Session I Stowell-Orbison/Surgical Pathology/Autopsy Awards Poster Session # 206, Monday Morning