The relationship between lymphocyte subtypes distribution and biliary cirrhosis in biliary atresia patients.

BACKGROUND & AIMS: Biliary cirrhosis progression varies in biliary atresia (BA) patients after hepatoportoenterostomy. Previous studies have suggested that BA pathogenesis may be related to immune dysregulation. This study evaluates the relationship between cirrhosis severity and lymphocyte subtype distribution in patients with BA after undergoing hepatoportoenterostomy.

METHODS

A total of 67 BA patients (29 males and 38 females, mean age: 10.1 years) with hepatoportoenterostomy were enrolled in this study. We assessed the liver stiffness measurement (LSM) by transient elastography, blood lymphocyte subtypes analysis, and serum cytokines. We analyzed the relationships between LSM and immune profiles.

RESULTS

BA subjects with significant biliary cirrhosis (LSM ≥25 kPa) have higher fraction of CD3-CD19+ B lymphocyte (p = 0.004) and lower fraction of CD3+CD8+ T lymphocyte, γδT lymphocyte and CD3-CD16+CD56+ NK lymphocyte (p = 0.02, 0.01 and 0.001, respectively) than BA patients with LSM <25 kPa. BA subjects with LSM ≥25 kPa have lower Th1 lymphocytes among CD4+ T lymphocytes than others (p = 0.007). Among CD8+ T lymphocytes, higher Naïve cytotoxic T lymphocyte fraction and lower fraction of central memory cytotoxic T lymphocyte and effector memory cytotoxic T lymphocyte were observed in BA subjects with LSM ≥25 kPa (p = 0.005, 0.004, and 0.002, respectively). The LSM of BA subjects is positively correlated with serum interleukin-10 and interferon-γ (p = 0.03 and 0.046, respectively)

CONCLUSIONS

Our study demonstrated that the progression of liver cirrhosis in BA patients after hepatoportoenterostomy is significantly correlated to the component and distribution of immune profiles.