Role of Mast Cells in Assessing the Degree of Fibrosis in Histopathological Scoring of Extrahepatic Biliary Atresia.

INTRODUCTION

Extrahepatic biliary atresia (EHBA) is a fibroinflammatory disorder characterised by the fibrosed development of extrahepatic biliary ducts, resulting in cholestatic jaundice. Inflammation and inflammatory cytokines have been implicated as causative factors in the development of fibrosis. The disease, in essence, is a rare entity with an incidence varying from 1 in 5000 to 1 in 18,000 live births in India. The role of inflammatory cells, such as mast cells, in causing fibrosis in liver biopsies is being researched in recent times to determine their impact on patient prognosis.

AIMS AND

OBJECTIVES

The aim of the study was to assess the number of mast cells in cases diagnosed as EHBA and correlate that with the degree of fibrosis and other histopathological findings in order to indicate the role of mast cells as fibrogenic inflammatory mediator.

MATERIAL AND

METHODS

The department of pathology carried out a retrospective observational study on 7 cases of suspected EHBA, in samples received from cases with ages from 8 weeks to 2 years, which were sent to the lab from the department of pediatric surgery. Extrahepatic biliary atresia was confirmed by histopathological examination of the liver biopsy specimen and intraoperative cholangiography taken during Kasai's portoenterostomy. Multiple stains were done on sections, and the common scoring systems mentioned by Lee.WS and Russo P in their respective papers, were used in reporting the cases for EHBA. Fibrosis was scored as per the modified Scheuer's system.

RESULTS

All the cases showed similar findings with a fair number of mast cells, variable degree of fibrosis accompanying inflammation, biliary bile plugs and degeneration in the hepatocytes. A positive trend was noted between an increase in mast cells and the degree of fibrosis in the biopsies, which were reported out as EHBA. Eosinophils were present in all the cases analyzed.

CONCLUSION

Presence of Mast cells in all cases of EHBA with an increasing number of mast cells with a higher degree of fibrosis can be a potential or probable indicator of prognosis and scoring in EHBAs, however in view of the study being a pilot study with a small sample size, a larger cohort with control arm needs to be studied to confirm the findings of our study.