What are the diagnostic and treatment steps for HELLP syndrome?

Diagnosis and Treatment of HELLP Syndrome

The definitive management of HELLP syndrome requires immediate delivery after maternal stabilization, regardless of gestational age, as it represents a serious complication with significant maternal mortality risk of 2-24%. 1

Diagnostic Criteria for HELLP Syndrome

HELLP syndrome is diagnosed clinically based on the following laboratory findings:

• H (Hemolysis)

  • Evidence of microangiopathic hemolytic anemia on peripheral blood smear
  • Elevated lactate dehydrogenase (LDH) > 600 U/L
  • Decreased haptoglobin levels

• EL (Elevated Liver Enzymes)

  • Aspartate aminotransferase (AST) ≥ 70 U/L
  • Alanine aminotransferase (ALT) elevated (typically > 45 U/L)

• LP (Low Platelets)

  • Platelet count < 100,000/mm³

Clinical Presentation

• Right upper quadrant or epigastric pain (65% of cases)
• Nausea and vomiting (35%)
• Headache (30%)
• Malaise
• Jaundice (up to 40%)
• Hypertension (85% of cases)
• Proteinuria (common but not required)
• Weight gain and peripheral edema 1, 2

Diagnostic Steps

1. Laboratory testing:

   • Complete blood count with peripheral smear
   • Liver function tests (AST, ALT, LDH, bilirubin)
   • Coagulation studies (PT, aPTT, fibrinogen, fibrin degradation products)
   • Renal function tests
   • Urinalysis for proteinuria 3, 2

2. Abdominal imaging:

   • Perform abdominal imaging to rule out hepatic hemorrhage, infarct, or rupture 1

3. Differential diagnosis:

   • Acute fatty liver of pregnancy
   • Viral hepatitis
   • Cholecystitis
   • Thrombotic thrombocytopenic purpura
   • Hemolytic uremic syndrome
   • Immune thrombocytopenic purpura 4

Treatment Algorithm

1. Maternal Stabilization

• Control severe hypertension (SBP ≥160 mmHg and/or DBP ≥110 mmHg):

  • First-line medications: IV labetalol, IV hydralazine, or oral nifedipine
  • Target blood pressure: diastolic 85 mmHg, systolic 110-140 mmHg 3

• Seizure prophylaxis:

  • Administer magnesium sulfate
  • Continue for at least 24 hours postpartum 3

• Correct coagulopathy:

  • Transfuse platelets if count < 50,000/mm³, especially prior to cesarean section
  • Administer fresh frozen plasma to correct coagulopathy if needed
  • Transfuse whole blood or packed red cells if hemoglobin < 10 g/dL 3, 1

2. Delivery Planning

• Immediate delivery is the definitive treatment regardless of gestational age after maternal stabilization 1, 3

• Mode of delivery:

  • Vaginal delivery is preferable if the maternal condition is stable and cervix is favorable
  • If cervix is unfavorable, consider cervical ripening followed by induction
  • Cesarean section if maternal condition is deteriorating or there are signs of fetal distress 5

• Corticosteroids:

  • For gestational ages between 24-34 weeks, administer corticosteroids for fetal lung maturation
  • Single course: either 2 doses of 12 mg betamethasone 24 hours apart or 6 mg dexamethasone 12 hours apart 5, 3

3. Postpartum Management

• Intensive monitoring:

  • Continue close surveillance for at least 48 hours after delivery
  • Monitor in high dependency unit with central venous pressure, urinary output, blood pressure, ECG, and oxygen saturation 1, 5

• Laboratory monitoring:

  • Monitor platelet count and LDH as the best indicators of disease progression
  • Continue monitoring liver enzymes, renal function, and coagulation parameters
  • Laboratory abnormalities typically peak 24 hours after delivery and resolve over several days 5, 6

• Blood pressure control:

  • Continue antihypertensive medications as needed
  • Monitor blood pressure every 4-6 hours for at least 3 days
  • Taper antihypertensive medications slowly after days 3-6 3

Important Considerations

• HELLP syndrome occurs in 0.2-0.6% of all pregnancies and in 10-20% of cases with severe preeclampsia 5
• About 70% of cases develop before delivery, with the majority occurring between 27-37 weeks gestation 5
• HELLP syndrome can occur without typical signs of preeclampsia (hypertension and proteinuria) in up to 20% of cases 4
• Maternal mortality ranges from 2-24%, highlighting the importance of prompt diagnosis and treatment 1
• HELLP syndrome complicated by hepatic rupture or acute liver failure should prompt transfer to a transplant center for evaluation 1

Pitfalls to Avoid

• Delayed diagnosis: Consider HELLP syndrome in all pregnant women with upper abdominal pain, regardless of presence of preeclampsia symptoms 4
• Misdiagnosis: HELLP can mimic other conditions such as acute cholecystitis, viral hepatitis, or thrombotic microangiopathies 4, 7
• Inadequate monitoring: The syndrome may worsen rapidly, requiring close monitoring of laboratory parameters and maternal condition 7
• Prolonged conservative management: Delaying delivery can lead to increased maternal and fetal complications 6