Zieveʼs Syndrome: Autoimmune Hemolytic Anemia Associated with Alcoholic Liver Disease and Hyperlipidemia

Abstract

Purpose: Zieve syndrome is a poorly-understood entity reported mostly in the mid-1970s, and only rarely in the English literature aft er the mid-1990s. It has been associated with fatty liver/cirrhosis, severe upper-abdominal and right-upper quadrant pain, jaundice, hyperlipidemia, and hemolytic anemia. When excess alcohol ingestion is the proximate cause, the condition improves rapidly when alcohol consumption is stopped. Methods: A 55-year-old female with history of alcoholic liver disease and newly diagnosed alcoholic hepatitis presents with worsening right-upper quadrant pain, jaundice, fatigue, and 30 lb weight loss 6 weeks prior to presentation. She was previously diagnosed with alcoholic hepatitis and treated with methylprednisolone. Upon current presentation, the patient was found to a have a normocytic anemia with hemoglobin/hematocrit 7.4/21 and MCV 98. Her LDH was elevated to 1,097 and haptoglobin <10 with appropriate reticulocyte percent 9.1%. On review of peripheral smear, she was found to have microcytic spherocytes surrounding lymphocytes, consistent with autoimmune hemolytic anemia. Her direct antiglobulin test (DAT) was negative, but had positive cold agglutinins, which can be missed by DAT. A right-upper quadrant ultrasound with Dopplers was performed, fi nding cholelithiasis without cholecystitis, gallbladder polyp, no portal vein thrombus, and nodular hepatic contour. She initially treated with 3 days of prednisone 40 mg and IVIG 1g/kg/day without any response. With a MELD of 40 and worsening anemia, she was transferred to University of Maryland for liver transplant. A lipid panel was done prior to transfer, indicating total cholesterol of 200. Th e patient responded well to transplant and her symptoms resolved. Conclusion: Th is case demonstrated hyperlipidemia and hemolytic anemia consistent with Zieve’s syndrome. Hyperlipidemia may be due to excessive lipid mobilization or reduction in lipid clearing factors. Abnormally elevated serum lipids may contribute to red cell destruction. Both processes appear to be related to sequelae of acute liver failure due to alcohol abuse.