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, with the timing based on gestational age, maternal condition, and fetal status. 1

Diagnosis and Initial Assessment

• Diagnostic criteria for HELLP syndrome:

  • Hemolysis with increased LDH (>600 U/L)
  • Elevated liver enzymes (AST ≥70 U/L)
  • Low platelets (<100 x 10^9/L) 1, 2

• Essential laboratory tests:

  • 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) 1, 3

Management Algorithm

Pre-delivery Management

1. Blood pressure control:

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

2. Seizure prophylaxis:

   • Magnesium sulfate is the drug of choice 1

3. Management of coagulopathy:

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

4. Monitoring:

   • Central venous catheter for critically ill patients
   • Urinary catheter for hourly output monitoring
   • Avoid diuretic therapy (plasma volume already reduced) 1

Delivery Considerations

Immediate delivery is indicated if:

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

Mode of delivery:

• Vaginal delivery is preferable when possible
• Cesarean section if:
  • Maternal condition is deteriorating rapidly
  • Fetal distress is present
  • Expedited delivery needed and vaginal delivery not imminent 1

Anesthetic considerations:

• Regional anesthesia may be limited by coagulopathy
• If general anesthesia is required:
  • Use drugs with minimal hepatic/renal metabolism
  • Propofol preferred for induction (lacks active metabolites)
  • Consider difficult airway management 1, 4

For pregnancies <34 weeks:

• Single course of corticosteroid therapy for fetal lung maturation:
  • 2 doses of 12 mg betamethasone 24 hours apart, OR
  • 6 mg dexamethasone 12 hours apart 1, 2

Post-Delivery Management

• Continue close monitoring for at least 48 hours (HELLP typically peaks 24 hours post-delivery)
• Continue magnesium sulfate for at least 24 hours post-delivery
• Monitor:
  • Blood pressure changes
  • Laboratory parameters
  • Renal function
  • Neurological status 1, 2, 3

Complications Management

1. Hepatic complications:

   • Monitor for subcapsular hematomas, hepatic rupture, and hepatic failure
   • Perform abdominal ultrasound if symptoms suggest hepatic hematoma
   • Early referral to transplant center if signs of hepatic failure 1

2. Renal complications:

   • Monitor urine output and renal function tests 1

3. Pulmonary edema:

   • IV nitroglycerin (glycerol trinitrate): initial dose 5 mg/min IV infusion, gradually increasing every 3-5 minutes (max 100 mg/min) 1

4. Hypoglycemia:

   • Regular monitoring of maternal blood glucose 1, 3

Prevention in Future Pregnancies

• 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

Important Caveats

• HELLP syndrome occurs in 10-20% of cases with severe preeclampsia 1, 2
• About 70% of cases develop before delivery, with the remainder occurring within 48 hours after delivery 2, 5
• In 20% of cases, there is no hypertension, and 5-15% present with minimal or no proteinuria, making diagnosis challenging 6
• The risk of recurrence of hypertensive disease in subsequent pregnancies ranges from 27% to 48% 6
• Standard corticosteroid treatment for maternal HELLP syndrome (as opposed to fetal lung maturation) is of uncertain clinical value 2, 5