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

HELLP Syndrome: Workup and Management

HELLP syndrome requires immediate delivery if diagnosed at ≥34 weeks gestation, with maternal stabilization and consideration of expectant management at <34 weeks only at centers with maternal-fetal medicine expertise. 1

Diagnostic Workup

Laboratory Investigations

• Complete blood count with peripheral smear (to assess platelet count and hemolysis)
• Liver function tests (AST, ALT)
• Lactate dehydrogenase (LDH) >600 U/L (marker of hemolysis)
• Renal function tests
• Coagulation profile including fibrinogen (to evaluate for DIC)
• Uric acid
• Urinalysis for proteinuria

Diagnostic Criteria (Tennessee Classification System)

• Hemolysis with increased LDH (>600 U/L)
• Elevated liver enzymes (AST ≥70 U/L)
• Low platelets (<100 x 10⁹/L)

Management Algorithm

Immediate Stabilization

1. Blood pressure control for severe hypertension (>160/110 mmHg):

   • First-line agents in monitored setting:
     • IV labetalol: 10-20 mg initially, then 20-80 mg every 10-30 minutes
     • IV hydralazine: 5-10 mg every 15-30 minutes
     • Oral nifedipine
   • Target BP: 110-140/85 mmHg 1

2. Seizure prophylaxis with magnesium sulfate:

   • Loading dose: 4-5g IV over 15-20 minutes
   • Maintenance: 1-2g/hour continuous infusion
   • Monitor in high-dependency or intensive care setting for 24-48 hours 1

3. Correction of coagulopathy:

   • Platelet transfusion if count <50,000/mm³, especially prior to Cesarean section
   • Whole blood or packed red cells if hemoglobin <10 g/dL 1

Definitive Management Based on Gestational Age

≥34 weeks gestation:

• Immediate delivery is recommended 1, 2

<34 weeks gestation:

• Consider expectant management ONLY at centers with maternal-fetal medicine expertise
• Administer corticosteroids for fetal lung maturity:
  • 2 doses of 12 mg betamethasone 24 hours apart OR
  • 6 mg dexamethasone 12 hours apart 2
• Proceed with delivery if:
  • Maternal condition worsens
  • Progressive thrombocytopenia develops
  • Liver enzymes progressively worsen
  • Renal function deteriorates
  • Pulmonary edema develops
  • Neurological symptoms appear
  • Non-reassuring fetal status 1, 2

Mode of Delivery

• Vaginal delivery is preferable if feasible
• If cervix is unfavorable, consider cervical ripening followed by induction
• Cesarean section if indicated for obstetric reasons or maternal deterioration 2

Anesthetic Considerations

• Neuraxial anesthesia (spinal) is first choice for cesarean if platelet count is adequate and not rapidly declining
• General anesthesia with rapid sequence induction if contraindications to neuraxial anesthesia exist
• Be prepared for difficult airway management 3

Post-Delivery Care

• Continue close monitoring for at least 48 hours after delivery (HELLP often peaks 24 hours postpartum)
• Continue blood pressure monitoring every 4-6 hours for at least 3 days
• Taper antihypertensive medications slowly after days 3-6
• Follow-up within 1 week if still on antihypertensives at discharge 1

Common Pitfalls and Caveats

• Diagnostic challenges: HELLP may be confused with acute fatty liver of pregnancy, idiopathic thrombocytopenia, hemolytic uremic syndrome, or appendicitis 4
• Incomplete HELLP: Not all components may be present initially; condition can progress rapidly
• Postpartum HELLP: Can develop within 48-72 hours after delivery, requiring continued vigilance 4
• Laboratory monitoring: LDH and platelet count are the two best tests to monitor disease progression 5
• Multisystem complications: HELLP can affect multiple organ systems including liver, kidneys, brain, and coagulation system, requiring comprehensive monitoring

The management of HELLP syndrome requires a multidisciplinary approach with prompt recognition and treatment in tertiary care centers to optimize maternal and fetal outcomes 6.