What is the management approach for a pregnant individual diagnosed with HELLP (Hemolysis, Elevated Liver enzymes, and Low Platelet count) syndrome?

Management of HELLP Syndrome in Pregnancy

Women with HELLP syndrome should be delivered promptly once maternal coagulopathy and severe hypertension have been corrected, as this is the definitive treatment to prevent worse maternal outcomes. 1

Initial Assessment and Stabilization

Laboratory Evaluation

• Complete blood count with platelet count (thrombocytopenia <100×10^9/L requires special attention)
• Liver function tests (AST, ALT, LDH)
• Renal function tests (creatinine, BUN)
• Coagulation studies (fibrinogen levels to evaluate for DIC)
• Urinalysis and protein quantification
• Serum haptoglobin (most sensitive marker for hemolysis)

Immediate Management

1. Blood pressure control:

   • For non-severe hypertension (140-159/90-109 mmHg): Oral labetalol, nifedipine, or methyldopa 1
   • For severe hypertension (≥160/110 mmHg): Urgent treatment in monitored setting with:
     • IV labetalol: 10-20 mg initially, then 20-80 mg every 10-30 minutes (max 220 mg)
     • IV hydralazine: 5-10 mg every 15-30 minutes
     • IV nicardipine: Start at 5 mg/h, increase by 2.5 mg/h every 5-15 minutes (max 15 mg/h) 2

2. Seizure prophylaxis:

   • Magnesium sulfate administration:
     • Loading dose: 4-5g IV over 15-20 minutes
     • Maintenance: 1-2g/hour continuous infusion
     • Continue for at least 24 hours postpartum 1, 2

3. Platelet management:

   • Platelet transfusion for counts <100×10^9/L due to increased risk of abnormal coagulation 1
   • Particularly important before delivery or invasive procedures

Delivery Considerations

Timing of Delivery

• ≥34 weeks gestation: Immediate delivery after stabilization 1, 2
• <34 weeks gestation:
  • Prompt delivery once maternal coagulopathy and severe hypertension are corrected 1
  • If maternal condition permits and there are no signs of fetal compromise, consider brief delay (24-48 hours) for administration of corticosteroids for fetal lung maturity 1, 3

Mode of Delivery

• Vaginal delivery can be accomplished in most cases if maternal condition is stable 3
• Cesarean section considerations:
  • Indicated for standard obstetric indications or rapid maternal deterioration
  • If platelet count <50,000/mm³, consider platelet transfusion before surgery 3
  • General anesthesia may be safer than regional anesthesia with severe thrombocytopenia 1

Special Considerations

Corticosteroids

• For fetal lung maturity: High-dose dexamethasone or betamethasone for pregnancies <35 weeks 1
• For maternal treatment: Not recommended to improve maternal outcomes in HELLP syndrome 1

Monitoring for Complications

• Abdominal ultrasound if symptoms suggest hepatic hematoma (abdominal/epigastric/right shoulder pain) 1
• Higher vigilance for hepatic hemorrhage with platelet counts <20×10^9/L 1
• Monitor for signs of hepatic failure requiring transplantation (early referral to transplant center if suspected) 1

Level of Care

• Treatment should be in a tertiary care center with maternal-fetal medicine expertise 3
• Consider intensive care admission for severe cases with end-organ dysfunction 1

Postpartum Management

• Continue magnesium sulfate for at least 24 hours postpartum 2
• Monitor blood pressure at least every 4-6 hours for minimum 3 days postpartum 2
• Taper antihypertensive medications slowly after days 3-6 postpartum unless BP becomes low (<110/70 mmHg) 2
• Follow-up at 3 months to ensure resolution of hypertension, proteinuria, and laboratory abnormalities 2

Long-term Follow-up

• Annual medical review lifelong due to increased future cardiovascular risk 2
• Counseling regarding 27-48% recurrence risk in future pregnancies 4
• Preconception counseling and first-trimester screening in subsequent pregnancies 1

Prevention in High-Risk Women

• Low-dose aspirin (150 mg) before 16 weeks' gestation until 36 weeks in subsequent pregnancies 1
• Calcium supplementation (1.5-2g/day) for women with low calcium intake (<800 mg/day) 1, 2