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.
K70.10 – Alcoholic hepatitis without ascites
44047000 – Zieve syndrome
- 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