What Is Zieve Syndrome?
Zieve Syndrome is an acute clinical condition and is characterized by a triad of jaundice, hyperlipidemia, and hemolytic anemia that occurs secondary to alcohol-induced liver injury. Anemia is the most common symptom and finding in an alcoholic individual. The alcoholic individual always has liver dysfunction, poor nutrition, and ineffective erythropoiesis.The underlying cause is liver delipidization, this is different from alcoholic hepatitis. It is a more common condition and estimated to affect 1 in 1600 individuals, but its mechanism is poorly understood. Early diagnosis is important to understand unnecessary interventions and tests.
What Is Spur Cell Anemia and How Is It Different From Zieve Syndrome?
Spur cells (acanthocytes) are erythrocytes covered with spike-like projections that vary in length, width, and distribution. Their deformity is responsible for entrapment and destruction in the spleen. Spur cell anemia is an acquired form of hemolytic anemia caused by a structural abnormality of red blood cell membranes resulting in spiculated erythrocytes. These peculiarly shaped red blood cells are acanthocytes.
Spur cell anemia lacks a landmark feature of Zieve syndromes like fever and abdominal pain. The individuals have low levels of total cholesterol and lipoprotein as opposed to high levels in Zieve syndrome.
What Are the Signs and Symptoms of Zieve Syndrome?
The signs and symptoms of Zieve's syndrome are:
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Anemia.
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Jaundice (yellowish discoloration of the mucous membrane of skin and whites of the eye).
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Abdominal pain.
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Nausea.
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Vomiting.
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Chest pain.
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Blockage of blood vessels in the brain and heart.
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Increased blood pressure.
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Fever.
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Dark-colored urine:
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Weakness.
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Dizziness (range of sensations like woozy, weak, feeling faint and unsteady).
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Confusion.
What Are the Causes of Zieve Syndrome?
Zieve syndrome was first reported in 1958, and the individuals with Zieve syndrome predominantly presented with jaundice, hyperlipidemias, and hemolytic anemia. Elevated circulating lipids levels in the individuals led to alterations in erythrocyte membrane composition and led to an increased susceptibility to hemolysis. The underlying cause is liver delilation. This occurs distinctly from alcoholic hepatitis and may present simultaneously or later.
How Is the Diagnosis of Zieve Syndrome Done?
Diagnosis of Zieve syndrome is done by confirming cholestatic jaundice and hemolysis which is secondary to alcoholic liver disease. In addition to these, the following tests are done:
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Complete Blood Test: Blood analysis is done to look for abnormal values of blood contents.
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Serum Enzyme Levels: Serum levels are analyzed to help in diagnosis. Here serum enzymes are markers in determining muscle tissue breakdown in the event of trauma or rhabdomyolysis.
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Liver Function Test: Also known as the hepatic panel test, helps in getting information regarding the functionality of the liver. These tests include prothrombin, bilirubin levels, albumin, and others.
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Ultrasound: Imaging tests help in the diagnosis of the internal organ's diseased condition.
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CT Scan: Computed tomography scan is done by combining a series of x-rays and images created using computer technology. It provides detailed imaging of bones, blood vessels, and soft tissue.
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MRI: Magnetic resonance image, an imaging technique that uses strong magnetic fields and radio waves to produce detailed images of the body tissues and bones.
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Biopsy and Tissue Analysis: Minimal invasive technique, part of the tissue or the affected area is removed and analyzed in the laboratory for further detailed analysis.
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Bone Marrow Biopsy: This biopsy is done by removing a small sample of bone marrow that is present inside the bone for analysis. The analysis is done by doing a biopsy using a fine needle aspiration technique and getting the sample from the bone.
What Is the Treatment for Zieve Syndrome?
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Patients usually recover in 4 to 6 weeks of alcohol abstinence. Cessation of alcohol leads to the shifting of enormous amounts of lipids from plasma and liver to adipose tissue as lipases become excessively activated, which leads to a decrease in plasma triglycerides.
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Plasmapheresis - It is a method of removing blood plasma from the body by withdrawing blood, separating it into cells and plasma, and transfusing cells back to the bloodstream. This procedure is done to remove antibodies in treating autoimmune conditions. It is not necessary for all individuals for hypertriglyceridemia. To decrease the risk of complications from hypertriglyceridemia, individuals should be evaluated by a history of pancreatitis and intracerebral hemorrhage.
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Long courses of glucocorticoids are used, but the use of glucocorticoids is linked with an increased risk of morbidity and mortality and increased hospitalization.
Can Zieve Syndrome Be Fatal?
In most cases, Zieve syndrome goes unrecognized by most healthcare providers. When diagnosed with Zieve syndrome, most individuals recover within four to six weeks of alcohol withdrawal. But if the alcohol consumption continues and symptoms and illness may aggravate and potentially be capable of being fatal.
What Is the Differential Diagnosis of Zieve Syndrome?
The differential diagnosis of Zieve's syndrome are:
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Chronic Macrocytic Anemia Is Secondary to Vitamin Deficiency: A blood disorder that occurs when the bone marrow produces abnormally large red blood cells.
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Autoimmune Hemolytic Anemia: It is a rare immune disorder and occurs when the body mistakes red blood cells for foreign cells that attack the body’s own cells. This condition is highly manageable and can result in fatal if left untreated.
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Aplastic Anemia: A rare condition in which the body stops producing enough new blood cells, and develops due to bone marrow damage.
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Leukemia: A cancer of blood-forming tissues that hinders the body’s ability to fight infections.
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Acute Alcoholic Hepatitis: Inflammation of the liver that is caused by chronic drinking of alcohol. It can develop suddenly due to prolonged alcohol intake and quickly lead to liver failure and death.
Conclusion:
The true cause of Zieve syndrome among individuals with alcoholic liver disease is not known. It is believed that the syndrome is underdiagnosed and only a few cases are reported so far. It is more important to look for direct symptoms of hyperbilirubinemia presenting individuals in the context of alcoholic liver disease. Hyper triglycerides are always at an increased level in such individuals, so a regular check of cholesterol and triglycerides are required. No treatment is recommended for individuals with transient hemolytic anemia due to Zieve syndrome; only supportive treatment is advised by encouraging abstaining from alcohol consumption.
