2223 Zieve’s Syndrome: Case Report, Review of the Literature and Electronic Patient Database

Abstract

INTRODUCTION: Zieve’s syndrome (ZS) is rare condition of alcoholic hepatitis, hyperlipidemia and hemolytic anemia1. Clinicians need to be aware of ZS for several reasons: First, hemolysis increases unconjugated bilirubin levels, leading to false elevation of the Maddrey’s discriminant function (MDF), which may lead to the unnecessary prescription of steroids.2 Second, the anemia that occurs from hemolysis may be misinterpreted as a gastrointestinal bleed, leading to extra endoscopic procedures. Finally, since patients can present with fever and abdominal pain, diagnosis may be construed for other causes such as sepsis or acute cholecystitis.2-5 CASE DESCRIPTION/METHODS: A 39-year-old female with long-standing alcohol abuse had three successive admissions resulting in refractory ZS. We performed a review of the literature to assess other case reports of patients with ZS. A total of 38 patients were included, 8 in women and 30 in men. Patients’ age ranged from 28 to 65 years old (mean 45.6 years). Average total bilirubin was 9.12. Average hemoglobin was 9.15. Average total cholesterol was 486. Average LDH was 243. Most patients did not receive prednisolone and fully recovered with strict alcohol cessation. A retrospective analysis using the IBM Explorys database of over 63 million de-identified, unique patients in the United States revealed that of 17,990 patients with alcoholic hepatitis, only 80 had ZS. The odds of a patient with alcoholic hepatitis also having hemolytic anemia is 7. ZS is rare, only seen in 0.4% of patient’s with alcoholic hepatitis. DISCUSSION: Our patient was 39, younger than the average age of 45.6 years. She was also female, a gender seen in a minority of previously reported cases. She had a total bilirubin 3 times above average, and a hemoglobin 33% lower than average. Despite 12 units of packed red blood cells, prednisolone, and pursuit of inpatient/outpatient alcohol rehabilitation, she ultimately succumbed to her liver injury 6 weeks after initially presenting to the hospital. This is the first reported case of death from ZS. Clinicians should maintain a high index of suspicion for ZS in patients with alcoholic hepatitis and transfusion refractory anemia. Evaluating for hemolysis and hyperlipidemia are recommended. Alcohol abstinence is essential. There is no role for steroids. Early diagnosis is essential. Our goal is that increased case reporting will raise clinician awareness.