Digestive Diseases and Sciences (2018) 63:601–604
UNM CLINICAL CASE CONFERENCES
Hemolysis in Alcoholic Liver Disease: Zieve’s Syndrome
· Swathi Paleti
· Euriko Torrazza Perez
· Denis McCarthy
Published online: 30 January 2018
© Springer Science+Business Media, LLC, part of Springer Nature 2018
Keywords Zieve’s syndrome · Hemolysis · Alcohol · Alcoholic liver disease · Hepatitis · Maddrey’s discriminant function
A 46-year-old female with history of asthma, hyperlipi-
demia, and heavy alcohol intake was initially evaluated for
the complaint of abdominal pain for 1 week, described as
generalized, aching, constant, nonradiating, and associated
with lassitude, nausea, vomiting, and diminished appetite.
She also noticed darkening of her urine over the same time.
The patient denied any overt upper or lower gastrointestinal
bleeding and reported no changes in her mental status. The
patient had consumed about 1 pint of vodka daily for the past
8 years. She reported that her last alcoholic beverage was
taken 3 days prior to admission. She denied illicit drug use.
On examination, she looked jaundiced, with scleral icterus
and conjunctival pallor. There were multiple chest telan-
giectasia within the distribution of the superior vena cava.
Physical examination revealed a slightly distended abdomen
with mild generalized tenderness. The liver span was 16 cm
and was mildly tender to direct palpation; Murphy’s sign
was negative. The spleen was not palpable, but there was
dullness on percussion in the ninth intercostal space on deep
inspiration, anterior to the mid-axillary line (a positive Cas-
tell’s sign). The ﬂanks were full but there was no shifting
dullness or ﬂuid wave. Bowel sounds were normal.
Laboratory tests showed: hematocrit 22% (36–48%),
hemoglobin 7.1 g/dl (12–14 g/dl), mean corpuscular vol-
ume 115 ﬂ (80–101 ﬂ), reticulocyte count 11% (0.5–1.5%),
serum concentrations of aspartate aminotransferase 118 U/l
(17–59 U/l), alanine aminotransferase 82 U/l (17–59 U/l),
total bilirubin 12 mg/dl (0.2–1.3 mg/dl), indirect bilirubin
10.0 mg/dl, lactate dehydrogenase 400 U/l (100–190 U/l),
lipase 285 mg/dl (10–73 mg/dl), triglycerides 208 mg/dl
(< 150 mg/dl), cholesterol 275 mg/dl ethanol 65 mg/dl,
and direct anti-globulin test (Coombs test) was negative.
Serum haptoglobin was undetectable (20–240 mg/dl), and
international normalized ratio (INR) was 1.7 (0.5–1.1). A
peripheral blood smear showed spur cells, schistocytes, and
prominent macrocytosis. A stool test was negative for occult
blood. Abdominal ultrasound with Doppler ﬂow measure-
ment showed patent vasculature, increased liver echogenic-
ity, enlarged spleen, and minimal ascites.
The patient was diagnosed with Zieve’s syndrome (hemo-
lytic anemia associated with alcoholic liver disease) and was
provided supportive management with intravenous hydra-
tion, thiamine supplementation, and management of alcohol
withdrawal. The patient recovered and was discharged home
after 5 days. The serum aminotransferases and bilirubin con-
centrations fell gradually during the hospital stay (Figs. 1,
2). The patient had addiction counseling before discharge,
and hospital follow-up was arranged in the liver disease
clinic and in an outpatient rehabilitation program.
Zieve’s syndrome, an alcohol-related hemolytic anemia,
was ﬁrst termed by Leslie Zieve in 1958 at the 38th Annual
Session of the American College of Physicians . Zieve
described a triad of hemolytic anemia, transient hyperlipi-
demia, and jaundice in 20 patients with a history of heavy
alcohol intake that resolved following alcohol abstinence.
The hemolysis is generally mild, short-lived, and the jaun-
dice (due to unconjugated hyperbilirubinemia) is not severe.
Zieve’s syndrome is a rare clinical phenomenon with only
205 cases reported in the literature since its initial descrip-
tion. Nevertheless, given the widespread chronic consump-
tion of excessive alcohol, the condition is thought to be more
* Anas Gremida
Division of Gastroenterology and Hepatology, University
of New Mexico School of Medicine, MSC 10-5550, 1
University of New Mexico, Albuquerque, NM 87131, USA