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Case Reports
. 2019 Nov-Dec;9(6):749-752.
doi: 10.1016/j.jceh.2019.03.007. Epub 2019 Mar 27.

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

Madhumita Premkumar  1 , Devaraja Rangegowda  1 , Anand Kulkarni  1 , Tanmay Vyas  1 , Shivani Dudha  1 , Rakhi Maiwall  1
Affiliations
Case Reports

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

Madhumita Premkumar et al. J Clin Exp Hepatol. 2019 Nov-Dec.

Erratum in

Abstract

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.

Keywords: ALT, alanine transaminase; AST, aspartate transaminase; CD4 lymphocytopaenia; ERCP, endoscopic retrograde cholangiopancreatography; HIV, human immunodeficiency virus; ICL, idiopathic CD4+ lymphocytopenia; biliary drainage; portal pylephlebitis; recurrent pyogenic cholangitis.

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Figures

Figure 1
Figure 1
MRCP revealed multiple small discrete and confluent intrahepatic peribiliary abscesses with right ductal hepatolithiasis. A short stricture was noted at the primary confluence. Pancreatic head and uncinate process showed multiple specks of calcification with a mildly dilated main pancreatic duct. MRCP, magnetic resonance cholangiopancreatogram.
Figure 2
Figure 2
CT image showing the portal vein septic thrombus and biliary abscesses. The septic splenic infarct is also seen. CT, computed tomography.
Figure 3
Figure 3
Liver biopsy showed parenchymal disarray focal giant cell transformation, ballooning degeneration. Portal tracts showed neutrophilic cholangiolitis and few ductular bile plugs. IgG4 stain was negative, and there was depletion of CD 4 + T cells.
See this image and copyright information in PMC

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