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 A lipoprotein is a biochemical assembly whose primary function is to transport hydrophobic lipid (also known as fat) molecules in water, as in blood plasma or other extracellular fluids. They consist of a triglyceride and cholesterol center, sur ...

jaundice Jaundice, also known as icterus, is a yellowish or greenish pigmentation of the skin and sclera due to high bilirubin levels. Jaundice in adults is typically a sign indicating the presence of underlying diseases involving abnormal heme meta ...

(elevation of

unconjugated bilirubin Bilirubin (BR) (Latin for "red bile") is a red-orange compound that occurs in the normal catabolic pathway that breaks down heme in vertebrates. This catabolism is a necessary process in the body's clearance of waste products that arise from the ...

), and abdominal pain. The underlying cause is liver delipidization. This is distinct from alcoholic hepatitis which, however, may present simultaneously or develop later. 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.

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 Hemolysins or haemolysins are lipids and proteins that cause lysis of red blood cells by disrupting the cell membrane. Although the lytic activity of some microbe-derived hemolysins on red blood cells may be of great importance for nutrient acqu ...

such as lysolecithin. Changes in membrane lipid compositions such as increased

cholesterol Cholesterol is any of a class of certain organic molecules called lipids. It is a sterol (or modified steroid), a type of lipid. Cholesterol is biosynthesized by all animal cells and is an essential structural component of animal cell mem ...

and polyunsaturated fatty acid (PUFA) have been reported during the hemolytic phase.

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 Plasmapheresis (from the Greek language, Greek πλάσμα, ''plasma'', something molded, and ἀφαίρεσις ''aphairesis'', taking away) is the removal, treatment, and return or exchange of blood plasma or components thereof from and to the ...

to avoid complications associated with hypertriglyceridemia.

History

Zieve's syndrome was initially described in 1958. Dr. Leslie Zieve described patients with a combination of

alcoholic liver disease Alcoholic liver disease (ALD), also called alcohol-related liver disease (ARLD), is a term that encompasses the liver manifestations of alcohol overconsumption, including fatty liver, alcoholic hepatitis, and chronic hepatitis with liver fibrosis ...

, hemolytic anemia and hypertriglyceridemia.

References

External links

Alcohol and health Diseases of liver Hepatology Syndromes {{med-sign-stub