Zieve syndrome is the triad of hemolytic anemia, cholestatic jaundice, and transient hyperlipidemia in the setting of alcohol use disorder. The presentation of anemia is subacute and severe, differing from the chronic macrocytic anemia secondary to vitamin deficiency common in alcohol use disorder. The liver damage in Zieve syndrome also warrants different management from acute alcoholic hepatitis and has better prognosis with reversibility.
The syndrome is poorly recognized by many physicians and is likely underreported, with only approximately 200 published cases since its characterization by Leslie Zieve in 1958. Patients range in age from 20 to 60 years. There is not a clear difference in incidence by sex or apparent geographical predisposition.
Pathogenesis of Zieve syndrome is poorly understood, but the hemolysis is unlikely to be autoimmune mediated. It is suspected that the hemolysis is caused by dysregulated blood lipids interacting with red blood cells (RBCs) in the setting of alcohol-induced vitamin E deficiency, which destabilizes erythrocyte glutathione enzyme. Zieve syndrome leads to subacute, severe, normocytic anemia with hemolysis, which differs from the common etiology of chronic, macrocytic anemia secondary to folic acid and B12 deficiency.
Zieve syndrome
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Codes
ICD10CM:
K70.10 – Alcoholic hepatitis without ascites
SNOMEDCT:
44047000 – Zieve syndrome
K70.10 – Alcoholic hepatitis without ascites
SNOMEDCT:
44047000 – Zieve syndrome
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Last Reviewed:06/20/2018
Last Updated:10/18/2018
Last Updated:10/18/2018
Zieve syndrome