Acute-on-Chronic Liver Failure and Successful Liver Transplantation in Pyruvate Kinase Deficiency

Area of Science:

• Hepatology
• Hematology
• Transplantation

Background:

• Pyruvate kinase deficiency (PKD) is a rare inherited metabolic disorder.
• PKD can lead to various complications, including liver disease.
• Liver disease in PKD is often associated with chronic hemolysis and iron overload.

Observation:

• A 55-year-old male patient with PKD presented with acute-on-chronic liver failure.
• The patient experienced kidney failure and severe hyperbilirubinemia.
• Liver injury was attributed to recurrent cholangitis, cholestasis from hemolysis, and iron deposition.

Findings:

• The patient underwent a successful liver transplant.
• Post-transplant recovery was favorable.
• The case illustrates the complex mechanisms of liver damage in PKD.

Implications:

• Liver transplantation is a viable option for managing severe liver disease in PKD patients.
• Understanding PKD's impact on the liver is crucial for patient management.
• This case expands knowledge on rare PKD complications and their treatment.