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Hemolytic Anemia Associated with Red Cell Membrane Defects
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Zieves syndrome is an unusual and poorly understood condition occurring in patients with alcoholic liver disease. Spherocytes are present on blood smear examination. Hyperlipoproteinemia and jaundice are features of Zieve’s. Zieve’s must be distinguished from spur cell anemia (see below).
Zieve syndrome in acute alcoholic hepatitis
Published in Baylor University Medical Center Proceedings, 2020
Ted George Achufusi, Jasmine Sandhu, Japjot Chahal, Zachary Shepherd, Kanish Mirchia
Zieve syndrome is considered a rare sequelae of chronic liver disease. The mechanism behind the hemolysis is not entirely understood, but it is hypothesized that pyruvate kinase instability leaves red blood cells susceptible to hemolysis by circulating hemolysins. A shift in membrane lipid composition, including elevated cholesterol and polyunsaturated fatty acid levels, has been documented in the hemolysis phase of Zieve syndrome. However, it’s important to note that almost 50% of patients with Zieve syndrome have been found to have normal lipid profiles.2 It is hypothesized that deficiency of lipoprotein lipase leads to the transient hyperlipidemia seen in this particular disease. Also, it is not unusual for patients to present with concomitant acute pancreatitis as a result of the hyperlipidemia, which was also present in our patient. Review of the literature suggests that some consider pancreatitis to be an essential feature of Zieve syndrome.3 Another proposed mechanism behind the hemolysis is vitamin E deficiency, commonly seen in alcoholics, which in turn leads to erythrocyte glutathione deficiency.2,4
Multifactorial jaundice and pigmented choledocholithiasis secondary to warm autoimmune hemolytic anemia and alcoholic cirrhosis
Published in Baylor University Medical Center Proceedings, 2022
Colten Watson, Mazen Hassan, Grant Breeland
This case raises the question of whether the two disease processes are linked. There are several case reports of Zieve syndrome4 addressing hemolytic anemia in alcoholic cirrhosis with prolonged abstention from alcohol, but no reports have presented definitive research on the mechanism involved or examined if there are multiple etiologies for the manifestation of autoimmune hemolytic anemia in the setting of cirrhosis.