Zieve's Syndrome
Zieve's Syndrome: Overview
Definition
A rare condition characterized by the triad of alcoholic liver disease, hemolytic anemia, and hyperlipidemia. First described by Leslie Zieve in 1958.
Key Components
- Jaundice and alcoholic hepatitis
- Acute hemolytic anemia
- Transient hyperlipidemia
- Strong association with chronic alcohol abuse
Epidemiology
- Predominantly affects adults aged 30-55 years
- More common in males (M:F ratio approximately 4:1)
- True prevalence likely underestimated due to underdiagnosis
- Higher incidence in populations with high alcohol consumption
Clinical Features
Primary Symptoms
- Jaundice with marked elevation of bilirubin
- Both direct and indirect hyperbilirubinemia
- Yellow discoloration of skin and sclera
- Abdominal pain
- Right upper quadrant pain
- May mimic acute cholecystitis
- Symptoms of anemia
- Fatigue and weakness
- Shortness of breath
- Pallor
Associated Features
- Constitutional symptoms
- Low-grade fever
- Nausea and vomiting
- Weight loss
- Malaise
- Signs of chronic liver disease
- Spider angiomas
- Palmar erythema
- Hepatomegaly
Laboratory and Diagnostic Findings
Hematologic Abnormalities
- Hemolytic anemia
- Decreased hemoglobin and hematocrit
- Elevated reticulocyte count
- Increased Mean Corpuscular Volume (MCV)
- Spherocytes on peripheral blood smear
- Hemolysis markers
- Elevated LDH
- Low haptoglobin
- Elevated indirect bilirubin
Lipid Profile
- Marked hyperlipidemia
- Elevated triglycerides (often >1000 mg/dL)
- Increased total cholesterol
- Abnormal lipoprotein pattern
Liver Function Tests
- Elevated transaminases
- AST typically > ALT (2:1 ratio)
- Gamma-glutamyl transferase elevation
- Mixed hyperbilirubinemia
- Altered coagulation profile
Imaging Studies
- Abdominal ultrasound
- Assessment of liver structure
- Evaluation for cirrhosis
- Rule out other pathologies
- Additional imaging as needed to exclude other causes
Treatment Approach
Primary Interventions
- Alcohol cessation
- Complete abstinence essential
- Addiction counseling and support
- Prevention of withdrawal symptoms
- Nutritional support
- Vitamin B complex supplementation
- Folate replacement
- Adequate protein intake
- Management of hemolysis
- Blood transfusion if severely symptomatic
- Iron supplementation if indicated
Supportive Care
- Fluid and electrolyte management
- Prevention of complications
- Pain management if needed
Complications and Special Considerations
Potential Complications
- Acute complications
- Severe anemia requiring transfusion
- Acute liver failure
- Pancreatitis
- Chronic complications
- Progression of liver disease
- Development of cirrhosis
- Portal hypertension
Prognosis and Follow-up
Prognostic Factors
- Good prognosis with alcohol cessation
- Reversibility of hemolysis and hyperlipidemia
- Risk of recurrence with continued alcohol use
Follow-up Care
- Regular monitoring
- Complete blood count
- Liver function tests
- Lipid profile
- Long-term management
- Alcohol abstinence support
- Prevention of complications
- Regular health screenings
