S5744 Zieve's Syndrome: The Overlooked Triad

Introduction: First described by Dr. Leslie Zieve in 1957, Zieve’s syndrome is a rare condition typically seen in individuals with a history of alcohol abuse. Although the pathology is not yet completely understood, it is characterized by a complex triad of jaundice, hemolytic anemia, and transient hyperlipidemia. At first presentation, Zieve’s syndrome is often misdiagnosed as alcoholic hepatitis due to overlapping symptoms, patient populations, and lab abnormalities. However, hemolytic anemia is the key feature that distinguishes it from alcoholic hepatitis. Timely diagnosis of Zieve’s syndrome by clinicians is crucial in order to avoid delayed recognition and unnecessary treatments that contribute to potential complications and increased healthcare costs. Case Description/Methods: A 41-year-old man with a history of alcohol associated cirrhosis presented with nausea and vomiting for one week. Admission labs were notable for a hemoglobin of 8.6 g/dL, haptoglobin of less than 8 mg/dL. His total bilirubin was elevated 14.3 mg/dL with direct bilirubin of 3.4 mg/dL and a predominantly indirect bilirubinemia. Peripheral smear demonstrated slight RBC fragments. Lipid panel was significant for cholesterol of 361 mg/dL and LDL of 272 mg/dL. On day 2 of hospitalization, he required 1 unit of packed red blood cells for a hemoglobin of 6.7 g/d. His hemoglobin initially improved to 7.8 g/dL, however, he required an additional transfusion later that hospitalization. Of note, his PEth 2 weeks prior to admission was consistent with moderate alcohol intake. On discharge, the patient was provided with alcohol cessation counseling and a treatment plan. Three months later, he was seen in the clinic and found to have a PEth <10 consistent with no alcohol detection. His hemoglobin 1 month later had improved to 12.2. Discussion: This case illustrates a classic presentation for Zieve syndrome. Patients often present with hemolytic anemia and hyperbilirubinemia, It is important to recognize this disorder in cirrhotic patients and differentiate this from other life threatening causes of anemia and cholestasis such as alcohol associated hepatitis. Patients can show marked improvement of anemia upon cessation of alcohol.