Zieve's syndrome

Zieve's syndrome is an acute metabolic condition that can occur during withdrawal from prolonged heavy alcohol use. It is defined by hemolytic anemia (with spur cells and acanthocytes), hyperlipoproteinaemia (excessive blood lipoprotein), jaundice (elevation of unconjugated bilirubin), and abdominal pain.[1] The underlying cause is liver delipidization. This is distinct from alcoholic hepatitis which, however, may present simultaneously or develop later.

Zieve's syndrome
SpecialtyGastroenterology 

Diagnosis of Zieve's syndrome should be considered in patients with prolonged alcohol use (especially after an episode of binge drinking) with an elevation of unconjugated bilirubin and without obvious signs of gastrointestinal bleeding.[2]

Pathogenesis

The proposed mechanism of the characteristic haemolytic anaemia in Zieve's syndrome is due to alteration of the red cell metabolism, namely pyruvate kinase instability leaving them susceptible to circulating hemolysin such as lysolecithin.[3] Changes in membrane lipid compositions such as increased cholesterol and polyunsaturated fatty acid (PUFA) have been reported during the hemolytic phase.[4]

Diagnosis

The diagnosis is demonstrated by the triad of alcoholic hepatitis or cirrhosis, hemolytic anemia, and hyperlipidemia.

Treatment

Definitive treatment for Zieve's syndrome is alcohol cessation. Individuals with markedly elevated triglycerides, particularly with a history of pancreatitis or intracerebral hemorrhage, may require plasmapharesis to avoid complications associated with hypertriglyceridemia.[5]

History

Zieve's syndrome was initially described in 1958.[6] Dr. Leslie Zieve described patients with a combination of alcoholic liver disease, hemolytic anemia and hypertriglyceridemia.

References
  1. Mehta, AB; N McIntyre (2004). Oxford Textbook of Clinical Hepatology. Oxford University Press. pp. 1786–1787. ISBN 0-19-262515-2.
  2. Shukla, Sandhya; Sitrin, Michael (2015-07-09). "Hemolysis in Acute Alcoholic Hepatitis: Zieve's Syndrome". ACG Case Reports Journal. 2 (4): 250–251. doi:10.14309/crj.2015.75. ISSN 2326-3253. PMC 4508957. PMID 26203455.
  3. Melrose, W. D.; Bell, P. A.; Jupe, D. M.; Baikie, M. J. (1990-01-01). "Alcohol-associated haemolysis in Zieve's syndrome: a clinical and laboratory study of five cases". Clinical and Laboratory Haematology. 12 (2): 159–167. ISSN 0141-9854. PMID 2208946.
  4. Kunz, F.; Stummvoll, W. (1970-10-01). "The significance of plasma phospholipids in Zieve syndrome". Blut. 21 (4): 210–226. ISSN 0006-5242. PMID 5531666.
  5. Choudhry, Faiza; Kathawa, Jolian; Kerton, Kelsey; Farshadsefat, Seina; Piper, Marc (July 2019). "Zieveʼs Syndrome Presenting With Severe Hypertriglyceridemia". ACG Case Reports Journal. 6 (7): 1–3. doi:10.14309/crj.0000000000000133. PMC 6722365.
  6. Zieve, L (March 1958). "Jaundice, hyperlipemia and hemolytic anemia: a heretofore unrecognized syndrome associated with alcoholic fatty liver and cirrhosis". Annals of Internal Medicine. 48 (3): 471–476. doi:10.7326/0003-4819-48-3-471. PMID 13521581.


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