2358 Zieve's Syndrome: A Case of an Unusually Profound Hemolysis in the Setting of Alcoholic Liver Disease

Abstract

INTRODUCTION: Zieve’s Syndrome is a cause of hemolytic anemia in alcoholic liver disease, thought to arise from altered erythrocyte metabolism (pyruvate kinase instability) and hyperlipidemia (lysolecithin may trigger hemolysis). It is an under recognized syndrome, and often times leads to patients undergoing extensive clinical work ups. CASE DESCRIPTION/METHODS: A 73-year-old male with a history of alcohol abuse presented to the emergency room for acute jaundice associated with melenic stools, and dark urine. Physical exam was notable for dry mucous membranes, scleral icterus and conjunctival pallor, No oral ulcers, rashes or petechiae were found. Initial labs included a hemoglobin of 5.5G/DL, LDH 753IU/L, and a negative Coombs test. Based on his liver function tests, his R factor was 1.6, consistent with a cholestatic pattern of injury. Through his hospital course he had a rising alkaline phosphatase as high as 226 Units/L, and total bilirubin of 11.5 MG/DL. Triglycerides were elevated (289MG/DL) compared to prior lipid study. Abdominal ultrasound revealed an echogenic liver consistent with fatty infiltration. Lab workup was negative for HFE (hemochromatosis) mutations, autoimmune hepatitis, HIV, CMV, HCV, and EBV. Prior to coming to the hospital, the patient spent time out in the woods. Given risk of tick exposure, in the setting of hemolytic anemia, he was evaluated for tick borne illness. He was started on Doxycycline, and Atovaquone to empirically cover Lyme and Babesia, as well as Piperacillin-Tazobactam while blood and urine cultures were pending. Parasite studies and blood/urine cultures returned negative. His hemolytic anemia was refractory to 8U of packed red blood cells, and 1U of fresh frozen plasma. Eventually, the patient was diagnosed with Zieve's Syndrome. DISCUSSION: Zieve’s Syndrome is characterized by a triad of alcoholic steatohepatitis, jaundice, and transient hyperlipidemia. The challenging aspect of this case lies in the fact that these findings are often seen under the constellation of other diseases, leading patients to unnecessary tests and studies. Immediate management of the syndrome is comprised of supportive care, while definitive treatment is alcohol cessation.