Makindo Medical Notes

🍺 Zieve's Syndrome is a rare but important cause of acute haemolytic anaemia in heavy alcohol users. It presents with the triad of: jaundice, hyperlipidaemia, and alcoholic steatohepatitis. First described in 1957 by Dr. Leslie Zieve, it is often under-recognised in clinical practice.

📖 About

• A combination of alcoholic hepatitis, haemolytic anaemia, and hyperlipidaemia.
• Occurs most often in patients with heavy or chronic alcohol misuse.
• Frequently overlooked as jaundice is attributed to alcoholic hepatitis alone.

⚠️ Aetiology & Pathophysiology

• Exact mechanism of haemolysis remains unclear.
• 🧬 Red cell metabolism changes (e.g., pyruvate kinase instability) → erythrocytes become fragile.
• 💥 Circulating haemolysins such as lysolecithin contribute to haemolysis.
• 🩸 Abnormal lipid metabolism → altered RBC membrane composition (↑ cholesterol & polyunsaturated fatty acids) during haemolytic phase.
• Usually seen in the context of acute alcohol binge on a background of chronic liver disease.

🩺 Clinical Features

• Jaundice (mixed picture: haemolysis + hepatocellular injury).
• RUQ pain, tender hepatomegaly from alcoholic hepatitis.
• Fatigue, pallor, and symptoms of anaemia.
• Alcohol-related problems: withdrawal, malnutrition, neuropathy.

❓ Differentials

• Alcoholic liver disease without haemolysis.
• Other causes of haemolytic anaemia (autoimmune, G6PD deficiency, microangiopathic haemolysis).
• Viral hepatitis.

🧪 Investigations

• FBC: Low Hb, ↑ reticulocytes (suggests haemolysis).
• Blood film: Spherocytosis, polychromasia.
• LFTs: Mixed hepatitic picture (↑ bilirubin, ↑ AST/ALT, ↑ ALP).
• Haemolysis markers: ↑ LDH, ↓ haptoglobins, unconjugated hyperbilirubinaemia.
• Lipid panel: Hyperlipidaemia (can fluctuate with disease phase).

💊 Management (Supportive)

• 🎯 No disease-specific therapy – mainstay is supportive care.
• Hydration and correction of electrolyte/clotting abnormalities.
• Monitor and manage haemolysis.
• Treat alcohol withdrawal (Thiamine, Chlordiazepoxide).
• Prevent further alcohol intake – abstinence is key to prevent recurrence.
• Ensure adequate nutrition (folate, B12, iron as appropriate).

📌 Clinical Pearls

• Think of Zieve’s syndrome in an alcoholic patient with **anaemia + jaundice not fully explained by hepatitis**.
• Haemolysis and hyperlipidaemia may spontaneously resolve within weeks of abstinence.
• Important to distinguish from autoimmune haemolytic anaemia as steroids are not indicated here.

📚 References

• Zieve L. "Jaundice, hyperlipemia, and hemolytic anemia: a heretofore unrecognized syndrome associated with alcoholic fatty liver and cirrhosis." Ann Intern Med. 1958.
• British Society of Gastroenterology – Alcohol-related liver disease guidance.
• UpToDate: Zieve’s Syndrome.