S1589 Zieve’s Syndrome: A Clinical Triad, or Perchance a Quartet?

Abstract

INTRODUCTION: Zieve’s syndrome is a syndrome that presents with a triad of hemolytic anemia, jaundice, and hyperlipidemia secondary to alcohol use/alcohol-induced liver injury, hemolytic anemia being the hallmark feature. It was first described by Dr. Leslie Zieve in 1957. CASE DESCRIPTION/METHODS: A 29-year-old male with no medical history presented with acute epigastric abdominal pain radiating to his back. Pain is associated with nausea and vomiting. Social history is significant for heavy alcohol use but negative for smoking and illicit drug use. Vital signs stable on admission. Physical exam was uncovered epigastric tenderness. Labs significant for elevated lipase to 2145, elevated transaminase enzymes AST/ALT 163/209. TBILI 1.1 and DBILI 0.3. HIV negative, Ethanol < 10, IgG4 28.3 WNL, Triglycerides 62, HbG 15.8, Hct 47.8, RDW 15.6%. Abdominal ultrasound was negative for gallstones. CT abdomen/pelvis revealed findings consistent with pancreatitis. His acute pancreatitis was managed with IVF and pain control. Throughout the hospital course, his Hgb dropped from 15.8 to 12.3 and an unconjugated bilirubinemia was appreciated. TBILI went from 1.1 to 2.8 and Indirect BILI went from 0.8 to 1.9. Hemolysis labs were significant for LDH 1386, Haptoglobin < 20, Reticulocyte 3%, DAT Negative. Ceruloplasmin wnl, fibrinogen elevated to 563, direct coombs neg. Peripheral smear revealed immature granulocytes, no schistocytes, and occasional nucleated RBCs. DISCUSSION: Zieve syndrome is more common than originally perceived as it’s incidence is estimated at 1 in 1,600 admissions, but its mechanism is poorly understood. Zieve suggested transient hyperlipidemia, specifically Lysolecithin, caused RBC membrane disruption. Zieve’s study reported hyperlipidemia in 50% of patients. A follow-up study by Balcerzak et al. reported that transfused RBCs into patients with Zieve’s Syndrome hemolyzed. In Zieve’s study, pancreatitis was frequently suspected but rarely established as CT scans did not yet exist and amylase had poor specificity and sensitivity for acute pancreatitis. Timely recognition of this syndrome may help prevent unnecessary diagnostic or therapeutic interventions. Zieve’s syndrome and its association with pancreatitis should be suspected in patients with hemolytic anemia in the setting of acute alcohol intake without signs of gastrointestinal bleeding. Future studies should be considered to evaluate the association between Zieve's syndrome and pancreatitis.