Zieve syndrome presenting with lipaemia and treated by plasmapheresis
- 1 Division of Cardiology, Medizinische Klinik und Poliklinik I, LMU Klinikum, Munchen, Germany
- 2 Division of Nephrology, Medizinische Klinik und Poliklinik IV, LMU Klinikum, Munich, Germany
- 3 Division of Angiology, Medizinische Klinik und Poliklinik IV, LMU Klinikum, Munich, Germany
- Correspondence to Dr John Michael Hoppe; john.hoppe@med.uni-muenchen.de
Abstract
Zieve syndrome is a rare condition which occurs in patients with severe alcohol abuse. It is typically characterised by the triad of jaundice, haemolytic anaemia and transient hyperlipidaemia. In the following report, we present the case of a man in his 30s who was admitted to our emergency department with severe epigastric pain and signs of alcohol intoxication. Blood samples showed signs of severe hyperlipidaemia and jaundice. Due to massive hyperlipidaemia, laboratory measurements of triglycerides were impaired and the blood samples had a ‘yellowish’ and ‘creamy’ texture. In order to prevent pancreatitis, plasmapheresis was performed. Subsequently, triglyceride concentration dropped and the patient was discharged a few days later in significantly improved physical condition. In the following case report, we discuss plasmapheresis as a possible treatment for patients with severe Zieve syndrome in addition to conventional symptomatic therapy.
Background
Zieve syndrome occurs in patients with severe alcohol abuse and is classically characterised by the triad of jaundice, haemolytic anaemia and transient hyperlipidaemia.1 To the best of our knowledge, Zieve syndrome with severe hyperlipidaemia requiring plasmapheresis has only been described once.2
Case presentation
A man in his 30s presented to our emergency department with severe epigastric pain and signs of alcohol intoxication. The patient had a history of alcohol abuse for more than 15 years and suffered from chronic hepatitis C, acquired during previous intravenous drug abuse. Physical examination revealed epigastric pain on palpation. Abdominal sonography showed a hepatic steatosis grade II and sludge in the gallbladder but no signs of acute pancreatitis. The blood samples taken had a ‘creamy’ yellowish texture (figure 1A). Initial blood analysis revealed values of bilirubin 2.1 mg/dL, gamma-glutamyltransferase 1736 U/L, glutamic-pyruvic transaminase 182 U/L, glutamic oxaloacetic transaminase 457 U/L, lactate dehydrogenase (LDH) 543 U/L, haemoglobin 125 g/L and a blood alcohol level 2.26 g/L. In addition to LDH elevation, haptoglobin was below the detection limit (<0.10 g/L), indicating subclinical haemolysis. Due to severe hyperlipidaemia, most other blood measurements, including lipase, were impaired. Only by dilution and titration, lipid analysis was feasible, revealing highly elevated blood concentrations of total cholesterol (968 mg/dL) and triglycerides (5679 mg/dL).
(A) Patient’s blood sample containing a yellowish fluid with a ‘creamy’ texture, barely recognisable as blood. Due to severe hyperlipidaemia, laboratory measurements were impaired. (B) Yellow fatty plasma filtered by plasmapheresis.
Treatment
In the given clinical constellation of acute alcoholic steatohepatitis together with lipaemia and haemolysis, a diagnosis of Zieve syndrome was established. As Maddrey’s discriminant function was below 32 points, we decided against corticosteroid treatment in addition to supportive measures (alcohol abstinence, hydration and B vitamin supplementation). However, in view of the excessively elevated triglycerides, we decided to perform plasmapheresis, primarily aiming on the prevention of acute pancreatitis. Approximately 3 L of yellowish fatty fluids were filtered in exchange for 20% concentrated human albumin (figure 1B).
Outcome
After plasmapheresis, triglyceride concentration dropped to 2722 mg/dL and then further decreased during the next few days (figure 2). Five days after admission, epigastric pain had subsided completely and the patient was discharged in significantly improved physical condition without any further specific medication. The patient presented to the emergency room several months later for another medical emergency. At the time of admission, there were no signs of Zieve syndrome recurrence.
Triglyceride-concentration (triglyceride-Co) during patient’s stay. On day 3 after plasmapheresis (PP), triglyceride-Co dropped rapidly. Dashed line depicts the presumed triglyceride-Co as measurements were impaired.
Discussion
Zieve syndrome was first described by Leslie Zieve in 1958, and since then less than 150 cases have been published. Due to the rarity of this condition, the pathogenesis as well as the true incidence remain mostly uncertain.3 Some research suggests an alteration in red cell metabolism as possible pathogenesis.4 Although the mechanism remains unclear, recent research showed features of an acquired pyruvate kinase deficiency and increased autohaemolysis in red blood cells.4
We here present an unusual case of Zieve syndrome with lipaemia as the leading clinical sign. Correspondingly, excessively elevated values of serum triglycerides were measured. The patient suffered from alcoholic steatohepatitis, whereas haemolysis by means of icterus was not clinically evident. However, laboratory analysis revealed mild haemolysis.
To assess the patient’s prognosis with regard to the acute hepatitis, we calculated Maddrey’s discriminant function, although results should be viewed under reservation as bilirubin levels may be elevated in the situation of haemolysis. As Maddrey’s discriminant function was below 32 points, we decided against corticosteroid treatment in addition to supportive measures (alcohol abstinence, hydration and B vitamin supplementation). Furthermore, corticosteroids have—to the best of our knowledge—not yet been described as a therapy for Zieve syndrome but may be of importance for suppression of concomitant acute hepatitis.
Severe hypertriglyceridaemia increases the risk of acute pancreatitis,5 and plasmapheresis has been described as a possible treatment option in patients with hypertriglyceridaemia >1000 mg/dL in order to prevent acute pancreatitis.2 Given the severity of hypertriglyceridaemia in our patient, we decided to perform plasmapheresis (single session), together with the above-mentioned supplemental measures. As a result, we observed a progressive decrease of serum triglycerides over the next few days and a relief of symptoms. We are not able to provide the long-term follow-up of our patient, and outcome data regarding recurrence risk, morbidity and mortality are not available. In addition, treatment with plasmapheresis was based on very limited data. Indication should depend on severity of symptoms and clinical parameters of pancreatitis. Repeating plasmapheresis treatments until triglyceride levels drop below 1000 mg/dL may be a reasonable approach; however, in this case, we decided against further treatments due to the patient’s rapid recovery. Lastly, it must be assumed that the prognosis of patients suffering from Zieve syndrome mainly depends on whether complete alcohol abstinence is achieved or not.
Learning points
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Zieve syndrome is probably underdiagnosed in patients with severe alcohol abuse.
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This condition is characterised by the triad of jaundice, hyperlipidaemia and haemolytic anaemia.
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Blood samples can appear creamy or yellowish.
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Plasmapheresis may prevent pancreatitis in severe cases of Zieve syndrome.
Ethics statements
Patient consent for publication
Footnotes
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Contributors LES, JK, MC and JMH contributed to the implementation of the case and to the analysis of the results. LS wrote the manuscript with support from MC and JMH. All authors were directly involved in the patient’s care.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
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Competing interests None declared.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.
References
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