What is the treatment for HELLP (Hemolysis, Elevated Liver enzymes, and Low Platelet count) syndrome?

Treatment of HELLP Syndrome

Delivery is the definitive treatment for HELLP syndrome, with immediate delivery indicated if the condition occurs after 34 weeks gestation or if maternal/fetal conditions deteriorate. 1, 2

Immediate Management

1. Blood Pressure Control

   • Urgent treatment required when BP >160/110 mmHg in a monitored setting
   • First-line medications:
     • 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: diastolic 85 mmHg, systolic 110-140 mmHg 1

2. Magnesium Sulfate Therapy

   • Indicated for women with HELLP syndrome to prevent seizures
   • 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. Blood Product Management

   • Platelet transfusion recommended if count <50,000/mm³, especially prior to Caesarean section 3
   • Whole blood or packed red cells if hemoglobin <10 g/dL 3
   • Monitor fibrinogen levels to evaluate for potential DIC 1

Delivery Considerations

1. Timing of Delivery

   • Immediate delivery for:
     • HELLP syndrome at ≥34 weeks gestation
     • Progressive thrombocytopenia
     • Progressively abnormal renal or liver function
     • Pulmonary edema
     • Abnormal neurological features
     • Non-reassuring fetal status 1, 2
   • Conservative management may be considered in selected cases <34 weeks with stable maternal and fetal condition at centers with maternal-fetal medicine expertise 2

2. Mode of Delivery

   • Vaginal delivery is preferable when possible 2
   • If cervix is unfavorable, cervical ripening followed by induction of labor
   • Caesarean section if rapid delivery is indicated or vaginal delivery not feasible

3. Anesthetic Considerations

   • Neuroaxial block (spinal anesthesia) is first choice for Caesarean if only moderate, non-progressive thrombocytopenia 4
   • General anesthesia if contraindications to regional anesthesia exist:
     • Use drugs with minimal hepatic/renal metabolism (e.g., propofol) 3
     • Control intubation stress response in patients with severe hypertension 4
     • Prepare for potentially difficult airway 4

Monitoring and Supportive Care

1. Laboratory Monitoring

   • Complete blood count with peripheral smear
   • Liver enzymes (AST, ALT)
   • Lactate dehydrogenase (LDH)
   • Renal function tests
   • Coagulation studies
   • Lactate dehydrogenase and platelet count are best tests to monitor disease course 5

2. Fluid Management

   • Consider central venous catheter or pulmonary artery catheter in critically ill patients 3
   • Many patients are relatively hypovolaemic despite edema
   • Monitor urinary output hourly via catheter 3

3. Additional Considerations

   • Monitor blood glucose (hypoglycemia reported in HELLP) 3
   • Corticosteroids for fetal lung maturity if <34 weeks (single course) 2
   • Continue close surveillance for at least 48 hours after delivery as HELLP typically peaks 24 hours postpartum 2, 5

Important Caveats

• HELLP syndrome is a progressive condition with high risk of serious complications 2
• Do not attempt to distinguish mild versus severe disease clinically as all cases may rapidly deteriorate 1
• The condition rarely remits spontaneously and typically worsens with time 1
• Peak intensity of HELLP syndrome typically occurs 24 hours after delivery 5
• Extended atypical HELLP has been successfully treated with plasma exchange in some cases 5