What are the diagnostic criteria and management for HELLP (Hemolysis, Elevated Liver enzymes, and Low Platelet count) syndrome?

Diagnostic Criteria and Management of HELLP Syndrome

HELLP syndrome should be diagnosed based on the triad of hemolysis, elevated liver enzymes, and low platelets (<100,000/mm³), and requires immediate delivery if gestational age is ≥34 weeks or if maternal/fetal condition is deteriorating. 1

Diagnostic Criteria

HELLP syndrome is a severe manifestation of preeclampsia, occurring in 10-20% of cases with severe preeclampsia 1. The diagnosis requires:

1. Hemolysis - Confirmed by:

   • Peripheral blood smear examination
   • Elevated indirect bilirubin
   • Low serum haptoglobin levels 2

2. Elevated Liver Enzymes - Significant elevation in:

   • AST (Aspartate aminotransferase)
   • ALT (Alanine aminotransferase)
   • LDH (Lactate dehydrogenase) 1

3. Low Platelets - Platelet count below 100,000/mm³ 2

Additional laboratory tests recommended for comprehensive evaluation:

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

Management Algorithm

Initial Assessment and Stabilization

1. Maternal Monitoring

   • Frequent blood pressure measurements
   • Laboratory monitoring (CBC, liver enzymes, renal function)
   • Neurological status assessment
   • Hourly urine output monitoring via urinary catheter 1

2. Blood Pressure Control

   • Target: <160/110 mmHg to prevent cerebral hemorrhage 1
   • 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, repeat every 20 minutes (max 30 mg)
     • Caution with concurrent magnesium sulfate due to hypotension risk 1

3. Seizure Prophylaxis

   • Magnesium sulfate therapy 1

Delivery Considerations

Immediate delivery is indicated if:

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

Delivery method:

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

Platelet management for delivery:

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

Post-Delivery Care

• 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 for:
  • Blood pressure changes
  • Laboratory parameters
  • Renal function
  • Neurological status 1

Complications to Monitor

1. Hepatic Complications

   • Subcapsular hematomas
   • Hepatic rupture
   • Hepatic failure (consider early referral to transplant center if present) 1

2. Renal Complications

   • Monitor urine output and renal function tests 1

3. Metabolic Complications

   • Monitor maternal blood glucose for hypoglycemia 1

4. Pulmonary Complications

   • Pulmonary edema (treat with IV nitroglycerin if needed) 1

Prevention in Future Pregnancies

For women with prior HELLP syndrome:

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

Common Pitfalls

1. Delayed Diagnosis

   • HELLP syndrome may occur without typical preeclampsia symptoms (hypertension/proteinuria) 3
   • Patients may be misdiagnosed with non-obstetric conditions, delaying appropriate treatment 4

2. Inadequate Monitoring

   • Failure to continue monitoring for 48-72 hours post-delivery can miss worsening condition 1

3. Inappropriate Management

   • Conservative management beyond 34 weeks is controversial and potentially dangerous 5
   • Failure to transfer to tertiary care centers with appropriate expertise 5

Early recognition, prompt intervention, and delivery at the appropriate time are critical to improving maternal and fetal outcomes in HELLP syndrome.