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Zieve syndrome
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Zieve syndrome

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Contributors: Sung Dave Jeon, Jennifer J. Findeis-Hosey MD
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Synopsis

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.

Codes

ICD10CM:
K70.10 – Alcoholic hepatitis without ascites

SNOMEDCT:
44047000 – Zieve syndrome

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Chronic macrocytic anemia secondary to vitamin deficiency (eg, folate, vitamin B12)
  • Acute alcoholic hepatitis – Zieve syndrome will frequently present with abdominal pain, profound hyperbilirubinemia, hyperlipidemia, and hemolytic anemia, while alcoholic hepatitis will have a macrocytic anemia with less pronounced hyperbilirubinemia.
  • Autoimmune hemolytic anemia
  • Aplastic anemia
  • Leukemia

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Therapy

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References

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Last Reviewed: 06/21/2018
Last Updated: 10/19/2018
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Zieve syndrome
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Zieve syndrome : Hepatomegaly, Jaundice, ALT elevated, AST elevated, Hemolytic anemia, Hypertriglyceridemia, Hyperbilirubinemia
Copyright © 2018 VisualDx®. All rights reserved.