S5828 Zieve Syndrome: A Diagnostic Challenge in Alcohol-Related Liver Injury

Introduction: Zieve Syndrome is an underdiagnosed hepatic condition arising in the context of chronic alcohol use. It is a triad of anemia, jaundice, and Hyperlipidemia. The syndrome is thought to be a result of fat release from the liver as well as breakdown of red blood cells in the setting of excessive alcohol consumption. Case Description/Methods: A 37-year-old woman with a history of alcohol use disorder, reportedly in remission, presented with sudden onset of right upper quadrant (RUQ) pain and jaundice. Laboratory findings revealed normocytic anemia (hemoglobin 6.0 g/dL), direct hyperbilirubinemia (total bilirubin 21.4 mg/dL, direct bilirubin 15.9 mg/dL) and undetectable ethanol levels. Abdominal ultrasound showed no abnormalities. Patient was coagulopathic, with an international normalized ratio of 2.1, while her platelet count was within normal limits. Lactate dehydrogenase (LDH) was elevated at 442 U/L, and haptoglobin was low (<1 mg/dL). A direct Coombs test was negative. Peripheral blood smear revealed poikilocytosis with target and fragmented cells. Liver biopsy revealed severe macrovascular steatosis with steatohepatitis and bile duct injury, suggestive of biliary obstruction. Supportive care, including blood transfusions, was provided while further liver injury investigations were pursued, all of which were non-revealing. After 10 days, a phosphatidylethanol (PEth) test indicated moderate alcohol consumption despite the patient's prior claims of remission. A final diagnosis of Zieve's syndrome was made. The patient’s hemoglobin stabilized, and her clinical condition improved, leading to discharge. Discussion: Due to the overlapping symptoms with other hepatic conditions, Zieve Syndrome is commonly misdiagnosed. Reluctance from patients to disclose alcohol consumption further increases diagnosis difficulty, leading to extensive investigations. PEth testing, a biomarker for chronic alcohol use, can be utilized in such cases. Treatment is supportive and dependent upon alcohol cessation. Steroids are non-beneficial and rarely used. Early recognition is crucial to prevent unnecessary testing and ensure appropriate management.