Difficult to Treat Recurrent Pyogenic Cholangitis With Portal Pylephlebitis in the Setting of Idiopathic CD4+ Lymphocytopaenia

Recurrent pyogenic cholangitis (RPC) is a disease characterized by multiple strictures of the biliary tree, impaired biliary drainage, formation of intrahepatic biliary pigment stones and recurrent bouts of cholangitis. We report the case of a 39-year-old businessman with diagnosed chronic calcific pancreatitis, who presented to us with recurrent episodes of cholangitis, leading to portal pyaemia, and progressive liver failure, which could not be controlled despite adequate biliary drainage. The patient rapidly developed progressive liver failure and sepsis-related coagulation failure. He was also found to have idiopathic CD4+ T cell lymphocytopenia (ICL), which resulted in refractory sepsis and formation of metastatic abscesses in the lung and spleen. ICL is now recognised in patients with recurrent and difficult to treat opportunistic infections. The combination of RPC, sepsis and liver failure in the setting of an acquired immunosuppressed state makes this a unique management scenario.