What is the management of Hellp (Hemolysis, Elevated Liver enzymes, and Low Platelet count) syndrome?

Management of HELLP Syndrome

The definitive treatment for HELLP syndrome is prompt delivery, especially if gestational age is ≥34 weeks, maternal condition is deteriorating, there is severe thrombocytopenia, or evidence of fetal distress. 1

Diagnosis and Initial Assessment

HELLP syndrome is characterized by:

• Hemolysis (with increased LDH >600 U/L)
• Elevated liver enzymes (AST ≥70 U/L)
• Low platelets (<100,000/mm³) 1, 2

Clinical presentation often includes:

• Epigastric or right upper quadrant pain (cardinal symptom)
• Nausea and vomiting
• Hypertension (though absent in 20% of cases)
• Proteinuria (may be minimal or absent in 5-15% of cases) 1, 3

Management Algorithm

1. Maternal Stabilization

• Blood Pressure Control:

  • Target BP <160/110 mmHg to prevent cerebral hemorrhage
  • First-line medications:
    • IV labetalol: 10-20 mg initially, then 20-80 mg every 10-30 minutes (max 220 mg)
    • IV nicardipine for hypertensive crisis
  • Second-line option: Oral nifedipine 10 mg, repeatable every 20 minutes (max 30 mg)
  • Caution when using nifedipine with magnesium sulfate due to hypotension risk 1

• Seizure Prophylaxis:

  • Magnesium sulfate is the drug of choice
  • Continue for at least 24 hours post-delivery 1

2. Laboratory Monitoring

• Complete blood count with peripheral smear
• Liver function tests (AST, ALT, LDH)
• Coagulation studies (PT, PTT, fibrinogen)
• Renal function tests
• Blood glucose monitoring (hypoglycemia can occur) 4, 1

3. Blood Product Management

• Platelet transfusion if count <50,000/mm³ for delivery, especially if cesarean section is planned
• Whole blood transfusion if hemoglobin <10 g/dL 4, 1

4. Fluid Management

• Central venous catheter placement for critically ill patients
• Urinary catheter for hourly output monitoring
• Avoid diuretic therapy as plasma volume is already reduced 4, 1

5. Timing and Mode of Delivery

Immediate delivery is indicated if:

• Gestational age ≥34 weeks
• Worsening maternal condition
• Severe thrombocytopenia
• Evidence of fetal distress 1, 2

Delivery considerations:

• Vaginal delivery is preferable when possible
• Cesarean section if maternal condition is deteriorating rapidly, fetal distress is present, or delivery needs to be expedited
• For gestations <34 weeks, administer corticosteroids for fetal lung maturity 1, 2

6. Anesthetic Considerations

• Regional anesthesia may be limited by coagulation disturbances
• If general anesthesia is required:
  • Use drugs with minimal hepatic/renal metabolism
  • Propofol is preferred for induction due to lack of active metabolites
  • Control intubation response to prevent cerebral complications
  • Consider difficult airway management 4, 1, 5

7. Post-Delivery Care

• Close monitoring for at least 48 hours after delivery
• HELLP syndrome typically peaks 24 hours post-delivery
• Continue magnesium sulfate for at least 24 hours
• Maintain blood pressure control
• Monitor for complications:
  • Hepatic complications (subcapsular hematomas, hepatic rupture)
  • Renal failure
  • Pulmonary edema
  • DIC 1, 2

Special Considerations

Conservative Management

Conservative management (>48 hours) is controversial but may be considered in selected cases <34 weeks' gestation in a tertiary care center with close maternal and fetal monitoring. However, delivery should be performed if maternal condition worsens or signs of fetal distress occur. 1, 2

Corticosteroid Use

• For fetal lung maturity: Single course of corticosteroids recommended for pregnancies <34 weeks
• For maternal HELLP syndrome: Standard corticosteroid treatment is of uncertain clinical value
• High-dose treatment and repeated doses should be avoided due to potential adverse effects on the fetal brain 1, 2

Complications and Prevention

Common Complications

• Hepatic complications (subcapsular hematomas, hepatic rupture, hepatic failure)
• Renal failure
• Pulmonary edema
• DIC
• Cerebral hemorrhage 1

Prevention in High-Risk Women

• First-trimester screening for women with prior HELLP syndrome
• Aspirin prophylaxis (150 mg nightly) before 16 weeks' gestation
• Calcium supplementation (1.5-2g/day) for women with low calcium intake 1

Risk of Recurrence

• The frequency for repeated hypertensive disease in subsequent pregnancies ranges from 27% to 48% 3

Pitfalls to Avoid

• Delaying delivery when indicated
• Inadequate blood pressure control (target <160/110 mmHg)
• Failing to monitor for complications for at least 48 hours post-delivery
• Overlooking HELLP syndrome in patients without hypertension or significant proteinuria
• Neglecting to monitor blood glucose (hypoglycemia can occur) 4, 1