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Zieve's syndrome

Last edited: 4/15/2026

Overview

Zieve's syndrome is characterized by jaundice, hyperlipemia, and hemolytic anemia, often associated with obstructive jaundice and hemolysis secondary to hemolysis triggered by biliary tract manipulation or obstruction 1.

Diagnosis

  • Clinical Presentation: Jaundice, elevated serum lipids, and evidence of hemolysis (elevated indirect bilirubin, low haptoglobin, schistocytes on peripheral smear) 1.
  • Laboratory Tests:
    • - Elevated total and direct bilirubin - Increased serum cholesterol and triglycerides - Decreased haptoglobin and elevated LDH - Peripheral blood smear showing schistocytes and other red cell abnormalities 1.

    Management

  • Supportive Care: Management of hemolysis with supportive measures such as hydration and monitoring for complications 1.
  • Address Underlying Cause: Prompt resolution of biliary obstruction through appropriate surgical or endoscopic interventions 1.
  • Lipid Management: Consideration of lipid-lowering agents if hyperlipidemia is severe and contributing to clinical symptoms (specific dosing not detailed in available abstracts) 1.

    • Special Populations

  • Comorbidities: Caution advised in patients undergoing invasive procedures like hepatic angiography due to increased risk of shock and mortality 1.

    • Key Recommendations

  • Avoid invasive procedures such as hepatic angiography in patients with Zieve's syndrome due to heightened risk of severe complications including shock and death (Evidence: Weak) 1.
  • Prioritize the identification and urgent treatment of the underlying biliary obstruction to prevent exacerbation of hemolytic anemia (Evidence: Expert opinion) 1.
  • Implement supportive care measures including close monitoring of hemolysis markers and manage hyperlipidemia as clinically indicated (Evidence: Expert opinion) 1.

    • References

    1 Pickens S. Fatality after hepatic angiography in Zieve's syndrome. Postgraduate medical journal 1979. link

    Original source

    1. [1]
      Fatality after hepatic angiography in Zieve's syndrome. — Pickens S Postgraduate medical journal (1979)
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