Document Type
Conference Proceeding
Publication Date
5-3-2024
Abstract
Introduction: Zieve’s syndrome describes a triad of non-immune hemolytic anemia, jaundice, and transient hyperlipidemia found in patients with alcohol-use disorder/alcohol-associated liver disease. This case involves a 39-year-old female with suspected Zieve’s syndrome in the setting of severe alcohol-associated hepatitis. Case Description: A 39-year-old female with alcohol-use disorder presented to the emergency department with complaints of jaundice, lethargy, and confusion. On initial presentation, patient was anemic with a hemoglobin of 7.8 g/dL (12.1- 15.0 g/dL), ALP of 235 U/L (33-120 U/L), AST of 116 U/L (< 35 U/L), ALT of 41 (< 37 U/L) total bilirubin of 24.2 mg/dL (< 1.2mg/dL), direct bilirubin of >15 mg/dL (< 0.4mg/dL). Her blood alcohol level was negative. She was admitted due to concerns for alcohol-associated hepatitis. Her hospital course was complicated by frequent declines in hemoglobin requiring a total of six transfusions of packed red blood cells. No overt bleeding such as hematemesis or hematochezia was ever evident during these declines. Further hematologic workup revealed a normal glucose 6 phosphate dehydrogenase (G6PD), normal pyruvate kinase, elevated plasma hemoglobin of 150 mg/dL (< 30 mg/dL), decreased haptoglobin of < 8 mg/dL (40-250 mg/dL), and elevated lactate dehydrogenase (LDH) of 595 (100-240 U/L), elevated reticulocyte count of 213 bil/L (21-100 bil/L). Direct Coombs Test was negative. The combination of these labs illustrated a picture of non-immune hemolytic anemia. Patient’s course was further complicated by a persistent rise in her total bilirubin, eventually peaking at 30.6 mg/dL, and ascites which required paracentesis and fluid studies was consistent with spontaneous bacterial peritonitis (SBP). Given the presence of SBP, corticosteroid therapy was not initiated for alcohol-associated hepatitis. Lipid panel remained within normal limits on two separate occasions. Vitamin E level was < 2.5 mg/L (5.5 – 17.0 mg/L). During hospitalization, she tested positive for Influenza A H1N1, developed acute hypoxic respiratory failure, was intubated, and was transferred to the intensive care unit and later extubated. Her mental status during her hospital stay was frequently waxing and waning and never fully recovered. The patient was eventually discharged home with hospice and her hemoglobin levels remained stable after supportive blood transfusions during her stay. Discussion: Zieve’s syndrome is an underdiagnosed cause of anemia in patients with alcohol-associated liver disease. There are multiple proposed mechanisms to explain the pathophysiology behind the red cell lysis seen in this disorder. One leading hypothesis suggests the red cell membrane becomes unstable due to vitamin E deficiency leading to disordered pyruvate kinase. Importance of early recognition of this syndrome is essential as treatment revolves around strict abstinence from alcohol.
Recommended Citation
Kiryakoza M, Ozturk NB, Skrzynski J. Non-immune hemolytic anemia in the setting of alcohol-associated hepatitis: Zieve's syndrome. Presented at: American College of Physicians Michigan Chapter and Society of Hospital Medicine Michigan Chapter 2024 Resident and Medical Student Day; 2024 May 3; Troy, MI
Comments
American College of Physicians Michigan Chapter and Society of Hospital Medicine Michigan Chapter 2024 Resident and Medical Student Day, May 3, 2024, Troy, MI